MedWorm: Huntington's Disease

Huntington Hill Installs Toshiba Aquilion Premium CT System

Health News from Medical News Today — Fri, 06 Nov 2009 06:00:00 +0100

Today's high-volume health care facilities require premium diagnostic imaging equipment that can image patients faster, with the best possible diagnostic accuracy. Huntington Hill Imaging Center has installed Toshiba America Medical Systems, Inc.'s Aquilion® Premium CT system, to enable them to meet the demands of their growing business. (Source: Health News from Medical News Today)

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Huntington Hill Installs Toshiba Aquilion Premium CT System

MRI / PET / Ultrasound News From Medical News Today — Fri, 06 Nov 2009 06:00:00 +0100

California imaging center deploys Toshiba's Aquilion CT

Health Imaging News — Wed, 04 Nov 2009 16:45:12 +0100

Huntington Hill Imaging Center in Pasadena, Calif., has installed Toshiba America Medical Systems' Aquilion Premium CT system. (Source: Health Imaging News)

California imaging center deploys Toshiba's Aquilion CT

Health Imaging News — Wed, 04 Nov 2009 16:45:12 +0100

Huntington Hill Imaging Center in Pasadena, Calif., has installed Toshiba America Medical Systems' Aquilion Premium CT system. (Source: Health Imaging News)

Biomarkers in Huntington's and Parkinson's Disease

Annals of the New York Academy of Sciences — Tue, 03 Nov 2009 00:00:00 +0100

Parkinson's (PD) and Huntington's disease (HD) are chronic neurodegenerative conditions of the brain with a variety of clinical presentations including a disorder of movement and a range of nonmotor deficits. HD is genetic in origin and the causative gene and protein known, namely mutant Huntingtin, which leads to widespread early neuronal dysfunction and death throughout the brain. In contrast, the etiology of sporadic PD is unknown, and the pathology targets the nigrostriatal dopaminergic neurons with the formation of [alpha]-synuclein positive Lewy bodies. In both diseases, the ability to accurately diagnose the disease in the early stages and monitor progression over time remains a major challenge given the majority of the pathology is sited deep within the CNS. This challenge has gain...

Efficacy Of Gene Therapy In Mouse Models Of Huntington's Disease Shown By Caltech Researchers

Health News from Medical News Today — Mon, 02 Nov 2009 08:00:00 +0100

Researchers at the California Institute of Technology (Caltech) have shown that a highly specific intrabody (an antibody fragment that works against a target inside a cell) is capable of stalling the development of Huntington's disease in a variety of mouse models. (Source: Health News from Medical News Today)

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Caltech researchers show efficacy of gene therapy in mouse models of Huntington's disease

EurekAlert! - Medicine and Health — Fri, 30 Oct 2009 04:00:00 +0100

(California Institute of Technology) Researchers at the California Institute of Technology have shown that a highly specific intrabody (an antibody fragment that works against a target inside a cell) is capable of stalling the development of Huntington's disease in a variety of mouse models. "Gene therapy in these models successfully attenuated the symptoms of Huntington's disease and increased life span," notes Paul Patterson, the Anne P. and Benjamin F. Biaggini Professor of Biological Sciences. (Source: EurekAlert! - Medicine and Health)

Client and therapist views on exercise programmes for early-mid stage Parkinson's disease and Huntington's disease.

Disability and Rehabilitation — Fri, 30 Oct 2009 00:00:00 +0100

Conclusions. Therapists should work in collaboration with clients to evaluate their individual considerations, identify barriers to exercise and design a client-specific programme that is acceptable and feasible for the client. PMID: 19874213 [PubMed - as supplied by publisher] (Source: Disability and Rehabilitation)

[Cell and ex vivo gene therapy: advances in the treatment of central nervous system disorders.]

Revista de Neurologia — Thu, 29 Oct 2009 21:08:24 +0100

CONCLUSIONS. Ex vivo cell therapy and gene therapy have helped to expand our knowledge about plasticity and the mechanisms and factors that promote cell integration within the central nervous system. Although behavioural improvements have been reported in animal and human models, further work is still required on these studies to clear up a number of dubious points. Ex vivo cell therapy and gene therapy in the nervous system constitute an important methodological tool with therapeutic possibilities that deserve further study. PMID: 19859890 [PubMed - in process] (Source: Revista de Neurologia)

Nervous System Drug-by-design: Formulation May Slow Parkinson's, Alzheimer's, Huntington's

ScienceDaily Headlines — Wed, 28 Oct 2009 09:00:00 +0100

A researcher in Israel is "building" a new drug, L803-MTS, to treat a number of central nervous system diseases, and it also shows promise against Parkinson's, Huntington's and diabetes. (Source: ScienceDaily Headlines)

New TAU Formulation May Slow Down Parkinson's, Alzheimer's And Huntington's Diseases

Health News from Medical News Today — Wed, 28 Oct 2009 07:00:00 +0100

Working like an architect, Prof. Hagit Eldar-Finkelman of Tel Aviv University's Sackler School of Medicine is "building" a new drug, L803-MTS, to treat a number of central nervous system (CNS) diseases like Alzheimer's. In pre-clinical studies, it also shows promise against Parkinson's, Huntington's and diabetes. (Source: Health News from Medical News Today)

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Key Concepts for Estimating the Burden of Surgical Conditions and the Unmet Need for Surgical Care

World Journal of Surgery — Wed, 28 Oct 2009 06:59:17 +0100

Conclusions Methodology is described for estimating the burden of surgical conditions and unmet need for surgical care. Using this approach it will be feasible to estimate the global burden of surgical conditions and help clarify where surgery fits among other global health priorities. These methods need to be validated using population-based data. Content Type Journal ArticleDOI 10.1007/s00268-009-0261-6Authors Stephen Bickler, University of California at San Diego Division of Pediatric Surgery San Diego USADoruk Ozgediz, University of Toronto Division of Pediatric Surgery 555 University Avenue Toronto ON Canada M5G 1X8Richard Gosselin, University of California at Berkeley School of Public Health 50 University Hall #7360 Berkeley CA 94720-7360 USAThomas Weiser, Harva...

Microglial CB2 cannabinoid receptors are neuroprotective in Huntington's disease excitotoxicity

Brain — Tue, 27 Oct 2009 17:42:28 +0100

Cannabinoid-derived drugs are promising agents for the development of novel neuroprotective strategies. Activation of neuronal CB1 cannabinoid receptors attenuates excitotoxic glutamatergic neurotransmission, triggers prosurvival signalling pathways and palliates motor symptoms in animal models of neurodegenerative disorders. However, in Huntington's disease there is a very early downregulation of CB1 receptors in striatal neurons that, together with the undesirable psychoactive effects triggered by CB1 receptor activation, foster the search for alternative pharmacological treatments. Here, we show that CB2 cannabinoid receptor expression increases in striatal microglia of Huntington's disease transgenic mouse models and patients. Genetic ablation of CB2 receptors in R6/2 mice, that expres...

Genetic and pharmacological inhibition of calcineurin corrects the BDNF transport defect in Huntington's disease

Molecular Brain — Tue, 27 Oct 2009 00:00:00 +0100

Huntington's disease (HD) is an inherited neurogenerative disease caused by an abnormal expansion of glutamine repeats in the huntingtin protein. There is currently no treatment to prevent the neurodegeneration caused by this devastating disorder. Huntingtin has been shown to be a positive regulator of vesicular transport, particularly for neurotrophins such as brain-derived neurotrophic factor (BDNF). This function is lost in patients with HD, resulting in a decrease in neurotrophic support and subsequent neuronal death. One promising line of treatment is therefore the restoration of huntingtin function in BDNF transport. (Source: Molecular Brain)

A nervous system drug-by-design

EurekAlert! - Social and Behavioral Science — Mon, 26 Oct 2009 04:00:00 +0100

(American Friends of Tel Aviv University) Prof. Hagit Eldar-Finkelman of Tel Aviv University is "building" a new drug, L803-MTS, to treat a number of central nervous system diseases, and it also shows promise against Parkinson's, Huntington's and diabetes. (Source: EurekAlert! - Social and Behavioral Science)

Cross-cutting issues and future directions for the OCD spectrum

Psychiatry Research — Fri, 23 Oct 2009 15:49:39 +0100

Abstract: The research planning agenda for DSM-V examined possible similarities in phenomenology, comorbidity, familial and genetic features, brain circuitry, and treatment response between obsessive-compulsive disorder (OCD) and several related disorders that are characterized by repetitive thoughts or behaviors. Such data support a re-examination of the DSM-IV-TR classification of OCD and the anxiety disorders, with possible inclusion of a group of obsessive-compulsive spectrum disorders (OCSDs) in DSM-V. Various disorders were systematically examined for inclusion in such a grouping, and later a smaller number were determined to meet threshold criteria for inclusion in the OCSDs. The disorders that were originally examined included OCD, obsessive-compulsive personality disorder (OCPD), ...

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The hOGG1 Ser326Cys polymorphism and Huntington's disease.

Toxicology — Fri, 23 Oct 2009 00:00:00 +0100

Authors: Coppedè F, Migheli F, Ceravolo R, Bregant E, Rocchi A, Petrozzi L, Unti E, Lonigro R, Siciliano G, Migliore L Increasing evidence supports a role for oxidative DNA damage and impaired DNA repair mechanisms in the pathogenesis of age related neurodegenerative diseases. Within this context there is a current interest in the understanding of the role played by polymorphisms of DNA repair genes in the inter-individual risk to develop neurodegenerative pathologies, as well as in the onset and the progression of disease symptoms. Particularly, somatic CAG repeat expansion of the gene encoding for huntingtin has been observed in tissues of patients affected by Huntington's disease (HD), including blood and brain. Recent evidence suggests that somatic CAG repeat expansion in HD c...

Cortical dopamine dysfunction in symptomatic and premanifest Huntington's disease gene carriers.

Neurobiology of Disease — Wed, 21 Oct 2009 00:00:00 +0100

Authors: Pavese N, Politis M, Tai YF, Barker R, Tabrizi SJ, Mason SL, Brooks DJ, Piccini P We used (11)C-raclopride PET, a marker of D2 dopamine receptor binding, and statistical parametric mapping (SPM) to localise cortical D2 receptor dysfunction in individual Huntington's disease (HD) gene carriers (16 symptomatic and 11 premanifest subjects) and assess its clinical significance. 62.5% of symptomatic HD patients and 54.5% of premanifest carriers showed cortical reductions in D2 binding. The most frequent decreases in cortical binding in individual HD subjects were seen in temporal and frontal areas. Symptomatic HD subjects with decreased cortical D2 binding had worse scores on neuropsychological tests assessing attention and executive functions than subjects without cortical dopamin...

Growth hormone and ghrelin secretion are associated with clinical severity in Huntington's disease

European Journal of Neurology — Tue, 20 Oct 2009 23:00:00 +0100

Conclusions: These findings suggest changes in the regulation of GH and ghrelin secretion dynamics in early stage HD patients that could become more prominent in the later stages of the disease. (Source: European Journal of Neurology)

A pilot study using nabilone for symptomatic treatment in Huntington's disease

Movement Disorders — Mon, 19 Oct 2009 23:00:00 +0100

Pilot study of nabilone in Huntington's disease (HD). Double-blind, placebo-controlled, cross-over study of nabilone versus placebo. Primary outcome, Unified Huntington's Disease Rating Scale (UHDRS) total motor score. Secondary measures: UHDRS subsections for chorea, cognition and behavior, and neuropsychiatric inventory (NPI). 44 randomized patients received either nabilone (1 or 2 mg) followed by placebo (n = 22), or placebo followed by nabilone (n = 22). Recruiting was straightforward. Nabilone safe and well tolerated, no psychotic episodes. Assessment of either dose of nabilone versus placebo showed a treatment difference of 0.86 (95% CI: -1.8 to 3.52) for total motor score; 1.68 (95% CI: 0.44 to 2.92) for chorea; 3.57 (95% CI: -3.41 to 10.55) for UHDRS cognition; 4.01 (95% CI: -0.11 ...

Gene expression changes in blood as a putative biomarker for Huntington's disease

Movement Disorders — Mon, 19 Oct 2009 23:00:00 +0100

Several studies demonstrated alterations of gene expression in blood in various neurological disorders including Huntington's disease (HD). Using microarray technology, a recent study identified a large number of significantly altered mRNAs in HD blood, from which a 12-gene set was selected as classifier for discriminating controls and HD patients. The aim of our study was to validate expression changes of these 12 genes in an independent cohort of HD patients and evaluate their sensitivity and specificity. Four different subject groups were included - patients with HD, Parkinson's disease (PD), acute ischemic stroke (AS) and healthy controls. Although the previous results were successfully validated, gene expression changes in HD blood partly overlapped with those observed in blood from P...

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Huntington disease: A tale of two genes

Neurology — Mon, 19 Oct 2009 20:01:47 +0100

(Source: Neurology)

Normal and mutant HTT interact to affect clinical severity and progression in Huntington disease

Neurology — Mon, 19 Oct 2009 20:01:47 +0100

Conclusions: Increasing CAG repeat size in normal HTT diminishes the association between mutant CAG repeat size and disease severity and progression in Huntington disease. The underlying mechanism may involve interaction of the polyglutamine domains of normal and mutant huntingtin (fragments) and needs further elucidation. These findings may have predictive value and are essential for the design and interpretation of future therapeutic trials. (Source: Neurology)

Phosphorylation of Threonine 3: IMPLICATIONS FOR HUNTINGTIN AGGREGATION AND NEUROTOXICITY [Protein Synthesis, Post-Translational Modification, and Degradation]

Journal of Biological Chemistry — Fri, 16 Oct 2009 13:36:19 +0100

Huntingtin (Htt) is a widely expressed protein that causes tissue-specific degeneration when mutated to contain an expanded polyglutamine (poly(Q)) domain. Although Htt is large, 350 kDa, the appearance of amino-terminal fragments of Htt in extracts of postmortem brain tissue from patients with Huntington disease (HD), and the fact that an amino-terminal fragment, Htt exon 1 protein (Httex1p), is sufficient to cause disease in models of HD, points to the importance of the amino-terminal region of Htt in the disease process. The first exon of Htt encodes 17 amino acids followed by a poly(Q) repeat of variable length and culminating with a proline-rich domain of 50 amino acids. Because modifications to this fragment have the potential to directly affect pathogenesis in several ways, we have ...

Abnormal morphology of peripheral cell tissues from patients with Huntington disease

Journal of Neural Transmission — Thu, 15 Oct 2009 18:08:13 +0100

Abstract We investigated the genotype-dependency of morphological abnormalities in peripheral cells from Huntington disease (HD) patients. Cell cultures derived from skin and muscle biopsies showed a different set of abnormalities depending on the genotype (i.e. heterozygous and homozygous for CAG mutations) and the tissue (i.e. fibroblasts and myoblasts). In general, homozygotes’ cell lines showed massive ultrastructural damage of specific cell organelles compared with age matched control. These consist of vacuolization, deranged crests and matrix found within giant mitochondria. In addition, enlarged endoplasmic reticulum and the occurrence of numerous autophagic vacuoles, which were similar to those occurring in neurons within affected brain areas, were described. Des...

Gladstone And Partners Receive $3.7 Million For Huntington's Disease Research

Health News from Medical News Today — Thu, 15 Oct 2009 07:00:00 +0100

The National Institutes of Health (NIH) has awarded a "Grand Opportunity" grant of $3.7 million to a consortium formed with the Gladstone Institute of Neurological Disease (GIND) and the Taube-Koret Center for Huntington's Disease Research to use stem cell technology to better understand Huntington's disease (HD) and to develop potential therapies. (Source: Health News from Medical News Today)

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Pituitary Adenylate Cyclase-Activating Polypeptide: Focus on Structure-Activity Relationships of a Neuroprotective Peptide.

Current Medicinal Chemistry — Wed, 14 Oct 2009 23:00:00 +0100

Authors: Bourgault S, Vaudry D, Dejda A, Doan ND, Vaudry H, Fournier A Pituitary adenylate cyclase-activating polypeptide (PACAP) is a 38-amino acid peptide that was initially isolated from hypothalamus extracts on the basis of its ability to stimulate the production of cAMP in cultured pituitary cells. Recent studies have shown that PACAP exerts potent neuroprotective effects not only in vitro but also in in vivo models of Parkinson's disease, Huntington's disease, traumatic brain injury and stroke. The protective effects of PACAP are based on its capacity to prevent neuronal apoptosis by acting directly on neurons or indirectly through the release of neuroprotective factors by astrocytes. These biological activities are mainly mediated through activation of the PAC1 receptor which is...

Research with transcranial magnetic stimulation in the treatment of aphasia

Current Neurology and Neuroscience Reports — Tue, 13 Oct 2009 10:45:07 +0100

We present language results from our rTMS studies as well as imaging results from overt naming functional MRI scans obtained before and after a series of rTMS treatments. Part 3 presents results from a pilot study in which rTMS treatments were followed immediately by constraint-induced language therapy. Part 4 reviews our diffusion tensor imaging study examining the possible connectivity of the arcuate fasciculus to different parts of Broca’s area (pars triangularis, pars opercularis) and to the ventral premotor cortex. The potential role of mirror neurons in the right pars opercularis and ventral premotor cortex in aphasia recovery is discussed. Content Type Journal ArticleDOI 10.1007/s11910-009-0067-9Authors Paula I. Martin, VA Boston Healthcare System Aphasia Research Center...

Gladstone and partners receive $3.7 million for Huntington's disease research

EurekAlert! - Biology — Tue, 13 Oct 2009 04:00:00 +0100

(Gladstone Institutes) The National Institutes of Health has awarded a "Grand Opportunity" grant of $3.7 million to a consortium formed with the Gladstone Institute of Neurological Disease and the Taube-Koret Center for Huntington's Disease Research to use stem cell technology to better understand Huntington's disease and to develop potential therapies. (Source: EurekAlert! - Biology)

FUS-Immunoreactive Intranuclear Inclusions in Neurodegenerative Disease

Brain Pathology — Mon, 12 Oct 2009 23:00:00 +0100

The objective of the present study was to determine the range of neurodegenerative disorders characterized by FUS-positive NIIs. Immunostaining for FUS revealed intense reactivity of NIIs in FTLD-IF and FTLD-UPS as well as in Huntington's disease, spinocerebellar ataxias 1 and 3, and neuronal intranuclear inclusion body disease. In contrast, there was no FUS staining of NIIs in inherited forms of FTLD-TDP caused by GRN and VCP mutations, fragile-X-associated tremor ataxia syndrome, or oculopharyngeal muscular dystrophy. In a cell culture model of Huntington's disease, NIIs were intensely FUS-positive. NII-bearing cells displayed loss of the normal diffuse nuclear pattern of FUS staining. This suggests that sequestration of nuclear FUS by NIIs may interfere with its normal nuclear localizat...

CORRECTION: Error in Table in: Progress and Challenges in RNA Interference Therapy for Huntington Disease

Archives of Neurology — Mon, 12 Oct 2009 19:51:48 +0100

(Source: Archives of Neurology)

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Error in Table in: Progress and Challenges in RNA Interference Therapy for Huntington Disease [Correction]

Archives of Neurology — Mon, 12 Oct 2009 19:51:48 +0100

(Source: Archives of Neurology)

[Reflection and Reaction] Neural grafts in Huntington's disease: viability after 10 years

Lancet Neurology — Sun, 11 Oct 2009 23:00:00 +0100

One of the major pathological features of Huntington's disease is the loss of projection neurons in the striatum, a subcortical structure on which cortical fibres converge and then relay mostly to the globus pallidus and the substantia nigra. Diagnosis depends on the presence of the dominant mutant Huntingtin gene (HTT), and clinical features include motor, intellectual, and psychiatric disorders. Although pharmacological drugs can alleviate symptoms, there is no effective cure and the disease progresses to dementia, a bedridden state, and death in about 15–20 years after onset. Despite this distressing outlook, results from animal studies have shown that anatomical and functional integration of fetal cell transplants in the host brain can sustain cognitive and motor recovery (see elsewh...

[Review] Epigenetic mechanisms in neurological diseases: genes, syndromes, and therapies

Lancet Neurology — Sun, 11 Oct 2009 23:00:00 +0100

Epigenetic mechanisms such as DNA methylation and modifications to histone proteins regulate high-order DNA structure and gene expression. Aberrant epigenetic mechanisms are involved in the development of many diseases, including cancer. The neurological disorder most intensely studied with regard to epigenetic changes is Rett syndrome; patients with Rett syndrome have neurodevelopmental defects associated with mutations in MeCP2, which encodes the methyl CpG binding protein 2, that binds to methylated DNA. Other mental retardation disorders are also linked to the disruption of genes involved in epigenetic mechanisms; such disorders include alpha thalassaemia/mental retardation X-linked syndrome, Rubinstein-Taybi syndrome, and Coffin-Lowry syndrome. Moreover, aberrant DNA methylation and h...

The Antiapoptotic Activity of Melatonin in Neurodegenerative Diseases.

CNS Neuroscience and Therapeutics — Fri, 09 Oct 2009 23:00:00 +0100

Authors: Wang X Melatonin plays a neuroprotective role in models of neurodegenerative diseases. However, the molecular mechanisms underlying neuroprotection by melatonin are not well understood. Apoptotic cell death in the central nervous system is a feature of neurodegenerative diseases. The intrinsic and extrinsic apoptotic pathways and the antiapoptotic survival signal pathways play critical roles in neurodegeneration. This review summarizes the reports to date showing inhibition by melatonin of the intrinsic apoptotic pathways in neurodegenerative diseases including stroke, Alzheimer disease, Parkinson disease, Huntington disease, and amyotrophic lateral sclerosis. Furthermore, the activation of survival signal pathways by melatonin in neurodegenerative diseases is discussed. P...

Fat City: W. Va. Town Braces for TV Show Depiction

FOXNews.com — Fri, 09 Oct 2009 12:21:01 +0100

Weary of being stuck with what they call the false label of America's unhealthiest city, Huntington residents are offering a wary welcome to a celebrity TV chef who hopes to help them shape up. (Source: FOXNews.com)

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Psychiatrist: It's a Wake-Up Call for 'Fat City'

FOXNews.com — Fri, 09 Oct 2009 06:36:53 +0100

If folks in Huntington, W. Va., aren't happy that their hometown is being called the Fattest City in America, then they can get off their sizable butts and do something about it, says psychiatrist Keith Ablow. (Source: FOXNews.com)

The effects of drug transporter inhibitors on the pharmacokinetics and tissue distribution of methotrexate in normal and tumor-bearing mice: a microdialysis study

Cancer Chemotherapy and Pharmacology — Fri, 09 Oct 2009 06:06:23 +0100

Conclusions This study revealed significant differences in the relative estimated PK parameters of the plasma, SC, peri-, and intratumoral zones. Additionally, this study demonstrated that the coadministration of MTX with CsA can enhance the intratumoral exposure levels of the drug, whereas coadministration of MTX with probenecid alone, or with a combination of probenecid and CsA, increases intratumoral half-life. Content Type Journal ArticleCategory Original ArticleDOI 10.1007/s00280-009-1146-yAuthors Shabnam N. Sani, Massachusetts College of Pharmacy and Health Sciences Department of Pharmaceutical Sciences 179 Longwood Avenue Boston MA 02115 USAKatherine Henry, Charles River Laboratories, In-Life Sciences, Preclinical Services 334 South St Shrewsbury MA 01545 USAM...

Dysregulation of coordinated neuronal firing patterns in striatum of freely behaving transgenic rats that model Huntington's disease.

Neurobiology of Disease — Tue, 06 Oct 2009 23:00:00 +0100

Authors: Miller BR, Walker AG, Fowler SC, von Hörsten S, Riess O, Johnson MA, Rebec GV Altered neuronal activity in the striatum appears to be a key component of Huntington's disease (HD), a fatal, neurodegenerative condition. To assess this hypothesis in freely behaving transgenic rats that model HD (tgHDs), we used chronically implanted micro-wires to record the spontaneous activity of striatal neurons. We found that relative to wild-type controls, HD rats suffer from population-level deficits in striatal activity characterized by a loss of correlated firing and fewer episodes of coincident spike bursting between simultaneously recorded neuronal pairs. These results are in line with our previous report of marked alterations in the pattern of striatal firing in mouse models of HD...

Implant-supported overdenture in an elderly patient with Huntington's disease

Gerodontology — Sun, 04 Oct 2009 23:00:00 +0100

Gerodontology 2009; doi:10.1111/j.1741-2358.2009.00343.xImplant-supported overdenture in an elderly patient with Huntington's disease Huntington's disease is a hereditary, progressive, neuro-degenerative disorder characterised by increasingly severe motor impairment, cognitive decline and behavioural manifestations leading to functional disability. Dyskinesia and hyperkinesia of the tongue and the peri-oral musculature make it impossible for the patient to wear a conventional complete denture, despite an adequate alveolar ridge. The present paper reports on a patient with Huntington's disease who was rehabilitated with a mandibular overdenture supported by two endosteal implants. One year follow-up examination showed that the prosthesis was stable and there was considerable improvement in ...

The Association of Adult Onchocerca volvulus with Lymphatic Vessels.

The Journal of Parasitology — Sun, 04 Oct 2009 23:00:00 +0100

Authors: Mackenzie CD, Huntington MK, Wanji SJ, Lovato RV, Eversole RR, Geary TG Immunocytochemical examination of onchocercal nodule tissues containing adult Onchocerca volvulus using immuno-markers for blood and lymphatic vessels (Factor VIII, D2-40, podoplanin, Prox-1 and Lyve1) shows a distinct pattern of distribution of these vessels within nodules. Blood vessels were commonly seen associated with organized lymphoid cellular aggregates in the both the outer and inner areas of the nodules. In contrast, the majority of the lymphatic vessel components were present in the central zones in close apposition to the adult parasites, or associated with microfilariae in the outer areas of the nodule. These findings suggest an intimate relationship between adult O.volvulus and lymphatic vess...

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Runners are baring their very soles

L.A. Times - Health — Sat, 03 Oct 2009 00:43:41 +0100

Barefoot runners, a small but growing number, say it reduces injuries and just feels better. "Ouch!" "Oooh!" "Oww!" "Omigod, that hurts!" Those grunts of pain and anguish weren't coming from us -- a group of 10 people running barefoot on a concrete pathway at Central Park in Huntington Beach early one recent Saturday morning. They were being emitted by a grimacing group of shoe-wearing, dog-walking women who were staring at us as we passed. (Source: L.A. Times - Health)

Coordination between Polymerase {beta} and FEN1 Can Modulate CAG Repeat Expansion [Dna: Replication, Repair, Recombination, and Chromosome Dynamics]

Journal of Biological Chemistry — Fri, 02 Oct 2009 15:40:25 +0100

The oxidized DNA base 8-oxoguanine (8-oxoG) is implicated in neuronal CAG repeat expansion associated with Huntington disease, yet it is unclear how such a DNA base lesion and its repair might cause the expansion. Here, we discovered size-limited expansion of CAG repeats during repair of 8-oxoG in a wild-type mouse cell extract. This expansion was deficient in extracts from cells lacking pol β and HMGB1. We demonstrate that expansion is mediated through pol β multinucleotide gap-filling DNA synthesis during long-patch base excision repair. Unexpectedly, FEN1 promotes expansion by facilitating ligation of hairpins formed by strand slippage. This alternate role of FEN1 and the polymerase β (pol β) multinucleotide gap-filling synthesis is the result of uncoupling of the us...

Activation of Gene Transcription by Heat Shock Protein 27 May Contribute to Its Neuronal Protection [Transcription, Chromatin, and Epigenetics]

Journal of Biological Chemistry — Fri, 02 Oct 2009 15:40:24 +0100

Heat shock proteins are up-regulated as a physiological response to stressful stimuli and generally function as molecular chaperones for improperly folded protein substrates. The small heat shock protein HSP27 (or HSPB1) has multiple cytoplasmic roles. HSP27 also can translocate to the nucleus in response to stress, but the functional significance of this nuclear distribution has not been elucidated. We have previously implicated HSP27 as a genetic modifier of spinocerebellar ataxia 17 (SCA17), a neurological disease caused by a polyglutamine expansion in the TATA-binding protein (TBP). Altered expression of HSP27 is also found in cell models of other polyglutamine diseases, including Huntington disease as well as SCA3 and SCA7. Here, we show that Hsp27, unlike Hsp70, is not detected in mu...

Remodeling of striatal NMDA receptors by chronic A(2A) receptor blockade in Huntington's disease mice.

Neurobiology of Disease — Thu, 01 Oct 2009 23:00:00 +0100

In conclusion, in vivo A(2A)R blockade induced a remodeling of NMDA receptors in the striatum of HD mice. Even though the functional relevance of the above effect remains to be fully elucidated, these results add further evidence to the modulatory role of A(2A)Rs in HD. PMID: 19804830 [PubMed - as supplied by publisher] (Source: Neurobiology of Disease)

Sleep patterns in patients with Huntington's disease and their unaffected first-degree relatives: a brief report.

Behavioral Sleep Medicine — Thu, 01 Oct 2009 09:00:02 +0100

In this study, by comparing the polysomnographic sleep patterns of HD patients and their unaffected relatives, identifying sleep traits more specifically related to the HD gene was attempted. The results corroborated previously reported findings of prolonged sleep latency and the virtual absence of nocturnal respiratory disturbances in early HD. Sleep latency in the HD patients positively correlated with the results of a screening test for frontal lobe dysfunction. Larger, more standardized studies will be needed to correlate genetic markers and sleep patterns in HD. PMID: 19787493 [PubMed - in process] (Source: Behavioral Sleep Medicine)

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Failure to thrive.

The Journal of Family Practice — Thu, 01 Oct 2009 00:00:00 +0100

Authors: Schmalz M, Boos K, Schmalz G, Huntington MK PMID: 19874733 [PubMed - in process] (Source: The Journal of Family Practice)

[The molecular bases of Huntington's disease: the role played by oxidative stress.]

Revista de Neurologia — Wed, 30 Sep 2009 23:00:00 +0100

CONCLUSIONS. Oxidative stress in patients with Huntington's disease may be used as an evolutionary-prognostic marker of both the disease and therapeutic effectiveness, as well as an interesting field of research for the development of new therapeutic strategies. PMID: 19816846 [PubMed - in process] (Source: Revista de Neurologia)

NAD(P)H oxidase contributes to neurotoxicity in an excitotoxic/prooxidant model of Huntington's disease in rats: Protective role of apocynin

Journal of Neuroscience Research — Tue, 29 Sep 2009 23:00:00 +0100

Intrastriatal injection of quinolinic acid (QUIN) to rodents reproduces some biochemical, morphological, and behavioral characteristics of Huntington's disease. NAD(P)H oxidase is an enzymatic complex that catalyzes superoxide anion (O2·-) production from O2 and NADPH. The present study evaluated the role of NAD(P)H oxidase in the striatal damage induced by QUIN (240 nmol/[mu]l) in adult male Wistar rats by means of apocynin (APO; 5 mg/kg i.p.), a specific NAD(P)H oxidase inhibitor. Rats were given APO 30 min before and 1 hr after QUIN injection or only 30 min after QUIN injection. NAD(P)H oxidase activity was measured in striatal homogenates by O2·- production. QUIN infusion to rats significantly increased striatal NAD(P)H oxidase activity (2 hr postlesion), whereas APO treatments decre...

3-nitropropionic acid-induced mitochondrial permeability transition: Comparative study of mitochondria from different tissues and brain regions

Journal of Neuroscience Research — Tue, 29 Sep 2009 23:00:00 +0100

The adult rat striatum is particularly vulnerable to systemic administration of the succinate dehydrogenase inhibitor 3-nitropropionic acid (3NP), which is known to induce degeneration of the caudate-putamen, as occurs in Huntington's disease. The aim of the present study was to compare the susceptibility of isolated mitochondria from different rat brain regions (striatum, cortex, and cerebellum) as well as from the liver, kidney, and heart to mitochondrial permeability transition (MPT) induced by 3NP and Ca2+. In the presence of micromolar Ca2+ concentrations, 3NP induces MPT in a dose-dependent manner, as estimated by mitochondrial swelling and a decrease in the transmembrane electrical potential. A 3NP concentration capable of promoting a 10% inhibition of ADP-stimulated, succinate-supp...

Possible New Huntington's Disease Treatment

About.com Mental Health — Mon, 28 Sep 2009 23:00:00 +0100

Stanford University Medical Center researchers have discovered a potential treatment for Huntington's disease. By enhancing the brain's natural protective response to the disease, researchers were able to alleviate the uncontrollable tremors and prolong the lives of mice with a neurological disorder that mimics Huntington's. Their finding suggests that a similar treatment strategy may be effective in humans. (Nature Medicine, Feb-2002) (Source: About.com Mental Health)

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Murine models of Huntington disease

Future Neurology — Mon, 28 Sep 2009 16:11:26 +0100

Future Neurology , September 2009, Vol. 4, No. 5, Pages 617-638. (Source: Future Neurology)

Pain reliever may be an anti-aging drug

Health News - UPI.com — Fri, 25 Sep 2009 20:45:43 +0100

HUNTINGTON, W.Va., Sept. 25 (UPI) -- Taking safe dosages of acetaminophen, a common pain reliever, may help prevent muscle loss due to aging, U.S. researchers suggest. (Source: Health News - UPI.com)

Chemical probes identify a role for histone deacetylase 3 in Friedreich's ataxia gene silencing.

Chemistry & Biology — Thu, 24 Sep 2009 23:00:00 +0100

Authors: Xu C, Soragni E, Chou CJ, Herman D, Plasterer HL, Rusche JR, Gottesfeld JM We recently identified a class of pimelic diphenylamide histone deacetylase (HDAC) inhibitors that show promise as therapeutics in the neurodegenerative diseases Friedreich's ataxia (FRDA) and Huntington's disease. Here, we describe chemical approaches to identify the HDAC enzyme target of these inhibitors. Incubation of a trifunctional activity-based probe with a panel of class I and class II recombinant HDAC enzymes, followed by click chemistry addition of a fluorescent dye and gel electrophoresis, identifies HDAC3 as a unique high-affinity target of the probe. Photoaffinity labeling in a nuclear extract prepared from human lymphoblasts with the trifunctional probe, followed by biotin addition through...

Huntington's Disease: Silencing a Brutal Killer.

Experimental Neurology — Thu, 24 Sep 2009 23:00:00 +0100

Authors: Pfister EL, Zamore PD PMID: 19786020 [PubMed - as supplied by publisher] (Source: Experimental Neurology)

Surgical techniques in ophthalmology series: retina and vitreous surgery with DVD

Graefe's Archive for Clinical and Experimental Ophthalmology — Thu, 24 Sep 2009 05:47:36 +0100

Content Type Journal ArticleCategory Book ReviewDOI 10.1007/s00417-009-1179-2Authors J. Sebag, VMR Institute Huntington Beach CA 92647 USA Journal Graefe's Archive for Clinical and Experimental OphthalmologyOnline ISSN 1435-702XPrint ISSN 0721-832X (Source: Graefe's Archive for Clinical and Experimental Ophthalmology)

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Apathy Is Not Depression in Huntington's Disease [REGULAR ARTICLES]

J Neuropsychiatry Clin Neurosci — Tue, 22 Sep 2009 23:00:00 +0100

Apathy and depression are common neuropsychiatric features of Huntington’s disease. The authors studied a group of 34 Huntington’s disease patients. In addition to the conventional classification according to DSM-IV criteria of depression, emphasis was put on a dimensional approach using scores on several different scales. Severe depression was found in 12% and severe apathy in 52% of all study patients. The authors found that apathy and depression are not related and are clearly distinct dimensions. Apathy was related to disease characteristics such as cognitive deterioration and functional decline, whereas depression was not. (Source: J Neuropsychiatry Clin Neurosci)

Thymic epithelial cells: the multi-tasking framework of the T cell "cradle"

Trends in Immunology — Tue, 22 Sep 2009 23:00:00 +0100

Authors: Alves NL, Huntington ND, Rodewald HR, Di Santo JP The thymus provides the anatomical "cradle" that fosters developing thymocytes. Thymic epithelial cells (TECs) are specialized cellular components that may be viewed as a multifunctional "frame" to nurture distinct stages of thymopoiesis. A symbiotic relationship between TECs and thymocytes exists because reciprocal interactions are required to achieve complete maturation of both cell types. Here, we propose that crucial instructive signals delivered by developing thymocytes negatively regulate functional attributes of immature TECs (including the expression of Delta-like 4 (DLL4) and interleukin-7 (IL-7)) that are required during early stages of thymopoiesis, while promoting the diversification of more mature TEC subsets. Thus...

Agendia Raises US $23 Million in Series E Financing

HSMN NewsFeed — Tue, 22 Sep 2009 17:36:10 +0100

Company to Present at UBS Global Life Sciences Conference on Sept. 23 HUNTINGTON BEACH, California and AMSTERDAM, September 22 (HSMN NewsFeed) -- Agendia, a world leader in molecular cancer diagnostics, today announced the successful closing of a US $... Diagnostics, Venture CapitalAgendia, MammaPrint (Source: HSMN NewsFeed)

Upper gastrointestinal findings in Huntington’s disease: patients suffer but do not complain

Journal of Neural Transmission — Mon, 21 Sep 2009 16:17:21 +0100

Abstract We investigated, retrospectively, the prevalence of gastroesophageal inflammation in patients with Huntington’s disease (HD) during 10 years in our center. We found a high prevalence of gastritis or esophagitis even in patients without complaints, indicating that gastrointestinal disease is more common in HD than patients’ complaints. There was no correlation with motor disturbances but with the duration and severity of HD. Influences from the disease itself as well as secondary mechanisms like malnutrition, medication and general disability may contribute. Content Type Journal ArticleCategory Basic Neurosciences, Genetics and Immunology - Short CommunicationDOI 10.1007/s00702-009-0310-1Authors Jürgen E. Andrich, Huntington-Center NRW, St. Josef Hospi...

The American Society Of Human Genetics Honors Dr. Huntington Willard As 2009 Allan Award Recipient

Health News from Medical News Today — Sat, 19 Sep 2009 07:00:00 +0100

The American Society of Human Genetics will present the 2009 William Allan Award to Huntington F. Willard, PhD, Director of the Institute for Genome Sciences & Policy (IGSP) at Duke University, in a formal ceremony at the Society's 59th Annual Meeting on October 23, 2009, in Honolulu, Hawaii. (Source: Health News from Medical News Today)

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Hsp70 molecular chaperones and Parkinson's disease

Biopolymers — Thu, 17 Sep 2009 23:00:00 +0100

Because over-expression of Hsp70 molecular chaperones suppresses the toxicity of aberrantly folded proteins that occur in Alzheimer's disease (AD), Parkinson's disease (PD), amyotrophic lateral sclerosis, and various polyQ-diseases (Huntington's disease and ataxias), Hsp70 is garnering attention as a possible therapeutic agent for these various diseases. Here I review progress in this fascinating field of molecular chaperones and neurodegeneration and describe our current understanding of the mechanisms by which Hsp70 protects cells from the PD-related protein called alpha-synuclein ([alpha]-syn). © 2009 Wiley Periodicals, Inc. Biopolymers, 2009 (Source: Biopolymers)

Structure and function of the molecular chaperone Hsp104 from yeast

Biopolymers — Thu, 17 Sep 2009 23:00:00 +0100

The molecular chaperone Hsp104 plays a central role in the clearance of aggregates after heat shock and the propagation of yeast prions. Hsp104's disaggregation activity and prion propagation have been linked to its ability to resolubilize or remodel protein aggregates. However, Hsp104 has also the capacity to catalyze protein aggregation of some substrates at specific conditions. Hence, it is a molecular chaperone with two opposing activities with respect to protein aggregation. In yeast models of Huntington's disease Hsp104 is required for the aggregation and toxicity of polyglutamine (polyQ), but the expression of Hsp104 in cellular and animal models of Huntington's and Parkinson's disease protects against polyQ and [alpha]-synuclein toxicity. Therefore, elucidating the molecular determ...

Prion Protein and Metal Interaction: Physiological and Pathological Implications.

Current Issues in Molecular Biology — Thu, 17 Sep 2009 23:00:00 +0100

Authors: Singh N, Das D, Singh A, Mohan ML Metal induced free radicals are important mediators of neurotoxicity in several neurodegenerative conditions such as Alzheimer's disease, Parkinson's disease, and Huntington's disease. Similar evidence is now emerging for prion diseases, a group of neurodegenerative disorders of humans and animals. The main pathogenic agent in all prion disorders is PrP-scrapie (PrP(Sc)), a beta-sheet rich isoform of a normal cell surface glycoprotein known as the prion protein (PrP(C)). Deposits of PrP(Sc) in the brain parenchyma are believed to induce neurotoxicity through poorly understood mechanisms. Recent reports suggest that imbalance of brain metal homeostasis is a significant cause of PrP(Sc)-associated neurotoxicity, though the underlying mechanisms ...

A Classification and Meta-analysis of Community-based Directly Observed Therapy Programs for Tuberculosis Treatment in Developing Countries

Journal of Community Health — Thu, 17 Sep 2009 12:34:31 +0100

Abstract In many developing countries, Directly Observed Therapy (DOT) for tuberculosis has been undertaken mainly in the clinic setting. However, clinic-based DOT may create a high patient load in already overburdened health facilities and increase barriers to care by requiring patients to travel to clinic frequently for therapy. Community-based DOT (CBDOT) may overcome some of these problems. This aims of this review are (a) to describe the main features of CBDOT programs, and (b) to compare features and outcomes of CBDOT programs that do and do not offer financial reward for CBDOT providers. Ten major features define CBDOT program structure and function. Programs that paid their CBDOT providers tended to differ from unpaid programs based on all of these features. CBDOT ...

The American Society of Human Genetics honors Dr. Huntington Willard as 2009 Allan Award recipient

EurekAlert! - Social and Behavioral Science — Thu, 17 Sep 2009 04:00:00 +0100

(American Society of Human Genetics) The American Society of Human Genetics will present the 2009 William Allan Award to Huntington F. Willard, PhD, Director of the Institute for Genome Sciences & Policy (IGSP) at Duke University, in a formal ceremony at the Society's 59th Annual Meeting on Oct. 23, 2009, in Honolulu, Hawaii. (Source: EurekAlert! - Social and Behavioral Science)

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Causes and significance of variation in mammalian basal metabolism

Wed, 16 Sep 2009 18:47:24 +0100

Abstract Mammalian basal metabolic rates (BMR) increase with body mass, whichs explains approximately 95% of the variation in BMR. However, at a given mass, there remains a large amount of variation in BMR. While many researchers suggest that the overall scaling of BMR with body mass is due to physiological constraints, variation at a given body mass may provide clues as to how selection acts on BMR. Here, we examine this variation in BMR in a broad sample of mammals and we test the hypothesis that, across mammals, body composition explains differences in BMR at a given body mass. Variation in BMR is strongly correlated with variation in muscle mass, and both of these variables are correlated with latitude and ambient temperature. These results suggest that selection alter...

Guidelines for intraoperative neuromonitoring using raw (analog or digital waveforms) and quantitative electroencephalography: a position statement by the American Society of Neurophysiological Monitoring

Journal of Clinical Monitoring and Computing — Wed, 16 Sep 2009 13:07:32 +0100

Conclusions This position paper summarizes commonly used protocols for recording and interpreting the intraoperative use of EEG. Furthermore, the American Society of Neurophysiological Monitoring recognizes this as primarily an educational service. Content Type Journal ArticleDOI 10.1007/s10877-009-9191-yAuthors Michael R. Isley, Orlando Regional Medical Center Intraoperative Neuromonitoring Department Orlando FL 32806 USAHarvey L. Edmonds, University of Louisville School of Medicine Department of Anesthesiology and Perioperative Medicine Louisville KY 40207-3633 USAMark Stecker, Marshall University School Of Medicine Neuroscience Department Huntington WV 25705 USA Journal Journal of Clinical Monitoring and ComputingOnline ISSN 1573-2614Print ISSN 1387-1307 (Sou...

Metabolic brain networks in neurodegenerative disorders: a functional imaging approach.

Trends in Neurosciences — Tue, 15 Sep 2009 23:00:00 +0100

Authors: Eidelberg D Network analysis of functional brain imaging data is an innovative approach to study circuit abnormalities in neurodegenerative diseases. In Parkinson's disease, spatial covariance analysis of resting-state metabolic images has identified specific regional patterns associated with motor and cognitive symptoms. With functional imaging, these metabolic networks have recently been used to measure system-related progression and to evaluate novel treatment strategies. Network analysis is also being used to characterize specific functional biomarkers for Huntington's disease and Alzheimer's disease. These networks have been particularly helpful in uncovering compensatory mechanisms in genetically at-risk individuals. Ongoing developments in network applications are likel...

The Pretravel Consultation.

American Family Physician — Mon, 14 Sep 2009 23:00:00 +0100

Authors: Bazemore AW, Huntington M PMID: 19817324 [PubMed - as supplied by publisher] (Source: American Family Physician)

Auxilio Announces Contract Renewal With Huntington Memorial Hospital

Medical News (via PRIMEZONE) — Mon, 14 Sep 2009 20:21:00 +0100

MISSION VIEJO, Calif., Sept. 14, 2009 (GLOBE NEWSWIRE) -- Auxilio, Inc. (OTCBB:AUXO) announced today that it has entered into an agreement for the renewal of its Document Image Management services with Huntington Memorial Hospital in Pasadena, California. Huntington Hospital has been a customer of Auxilio since April 2004 and the new agreement extends the relationship between the organizations for another three-year period. (Source: Medical News (via PRIMEZONE))

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AAFP-CIHI09 Introduction to Parasitology handout (Mark Huntington MD, PhD)

Family Medicine Digital Resources Library (FMDRL) Recently Uploaded — Sun, 13 Sep 2009 23:00:00 +0100

(Source: Family Medicine Digital Resources Library (FMDRL) Recently Uploaded)

Clinical Applications of Creatine Supplementation on Paediatrics.

Current Pharmaceutical Biotechnology — Sat, 12 Sep 2009 23:00:00 +0100

Authors: Athanasios E, Konstantina V, Paraskevi K, Nikolaos N Creatine plays a central role in energy metabolism and is synthesized in the liver, kidney and pancreas. In healthy patients, it is transported via the blood stream to the muscles, heart and brain with high and fluctuating energy demands by the molecule creatine transporter. Creatine, although naturally synthesized in the human body, can be ingested in the form of supplements and is commonly used by athletes. The purpose of this review was to assess the clinical applications of creatine supplementation on paediatrics. Creatine metabolism disorders have so far been described at the level of two synthetic steps, guanidinoacetate N-methyltransferase (GAMT) and arginine: glycine amidinotransferase (AGAT), and at the level of the...

AAFP-CIHI09 Introduction to Parasitology part 1 (Mark Huntington MD, PhD)

Family Medicine Digital Resources Library (FMDRL) Recently Uploaded — Sat, 12 Sep 2009 23:00:00 +0100

(Source: Family Medicine Digital Resources Library (FMDRL) Recently Uploaded)

AAFP-CIHI09 Introduction to Parasitology part 2 (Mark Huntington MD, PhD)

Family Medicine Digital Resources Library (FMDRL) Recently Uploaded — Sat, 12 Sep 2009 23:00:00 +0100

(Source: Family Medicine Digital Resources Library (FMDRL) Recently Uploaded)

The Basal Ganglia IX

Springer Biomedical Sciences titles — Tue, 08 Sep 2009 23:01:58 +0100

series: Advances in Behavioral Biology The International Basal Ganglia Society (IBAGS) furthers understanding of normal basal ganglia function and the pathophysiology of disorders of the basal ganglia. These disorders include Parkinson's disease, Huntington's disease, and schizophrenia. IBAGS triennial meetings bring together research scientists from all disciplines, as well as clinicians who are actively ... (Source: Springer Biomedical Sciences titles)

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[In Context] Book: Unravelling the mysteries of juvenile Huntington's disease

Lancet Neurology — Mon, 07 Sep 2009 23:00:00 +0100

Huntington's disease (HD) is one of the most common autosomal dominant disorders worldwide and one of the most devastating neurological diseases. The prevalence of HD is about 1 in 10 000 and it affects about 200 000 individuals in the Europe and about 100 000 in the USA. (Source: Lancet Neurology)

Meta-coexpression conservation analysis of microarray data: a "subset" approach provides insight into brain-derived neurotrophic factor regulation.

BMC Genomics - Latest articles — Mon, 07 Sep 2009 23:00:00 +0100

Conclusions: The study demonstrates the potential of the "subset" approach in co-expression conservation analysis for studying the regulation of single genes and proposes novel regulators of BDNF gene expression. (Source: BMC Genomics - Latest articles)

Protein Targets of Oxidative Damage in Human Neurodegenerative Diseases with Abnormal Protein Aggregates

Brain Pathology — Wed, 02 Sep 2009 23:00:00 +0100

Human neurodegenerative diseases with abnormal protein aggregates are associated with aberrant post-translational modifications, solubility, aggregation and fibril formation of selected proteins which cannot be degraded by cytosolic proteases, ubiquitin[ndash]protesome system and autophagy, and, therefore, accumulate in cells and extracellular compartments as residual debris. In addition to the accumulation of "primary" proteins, several other mechanisms are involved in the degenerative process and probably may explain crucial aspects such as the timing, selective cellular vulnerability and progression of the disease in particular individuals. One of these mechanisms is oxidative stress, which occurs in the vast majority of, if not all, degenerative diseases of the nervous system. The pres...

Stimulant Medications and Sudden Death

AAP Grand Rounds — Wed, 02 Sep 2009 23:00:00 +0100

(Source: AAP Grand Rounds)

Reduced calcineurin protein levels and activity in exon-1 mouse models of Huntington's disease: Role in excitotoxicity.

Neurobiology of Disease — Wed, 02 Sep 2009 23:00:00 +0100

Authors: Xifró X, Giralt A, Saavedra A, García-Martínez JM, Díaz-Hernández M, Lucas JJ, Alberch J, Pérez-Navarro E Calcineurin is a serine/threonine phosphatase involved in the regulation of glutamate receptors signaling. Here, we analyzed whether the regulation of calcineurin protein levels and activity modulates the susceptibility of striatal neurons to excitotoxicity in R6/1 and R6/1:BDNF+/- mouse models of Huntington's disease. We show that calcineurin inhibition in wild-type mice drastically reduced quinolinic acid-induced striatal cell death. Moreover, calcineurin A and B were differentially regulated during disease progression with a specific reduction of calcineurin A protein levels and calcineurin activity at the onset of the disease in R6/1:BDNF+...

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ADHD Guidance - Huntington Learning Center

Disabled World — Tue, 01 Sep 2009 21:40:36 +0100

National Institute of Mental Health recommends that students with ADHD get teaching in the form of guidance and understanding. Huntington Learning Center in Boca Raton, Florida provides both. (Source: Disabled World)

Breast cancer detection using automated whole breast ultrasound and mammography in radiographically dense breasts

European Radiology — Tue, 01 Sep 2009 16:47:52 +0100

Conclusion AWBU resulted in significant cancer detection improvement compared with mammography alone. Additional detection and the smaller size of invasive cancers may justify this technology’s expense for women with dense breasts and/or at high risk for breast cancer. Content Type Journal ArticleCategory UltrasoundDOI 10.1007/s00330-009-1588-yAuthors Kevin M. Kelly, Huntington-Hill Breast Center Huntington Memorial Hospital Pasadena CA USAJudy Dean, 1525 State St. Suite 102 Santa Barbara CA 93101 USAW. Scott Comulada, University of California Semel Institute Center for Community Health Los Angeles CA USASung-Jae Lee, University of California Semel Institute Center for Community Health Los Angeles CA USA Journal European RadiologyOnline ISSN 1432-1084Print IS...

Instrumenting the health care enterprise for discovery research in the genomic era [RESOURCES]

Genome Research — Mon, 31 Aug 2009 23:00:00 +0100

Tens of thousands of subjects may be required to obtain reliable evidence relating disease characteristics to the weak effects typically reported from common genetic variants. The costs of assembling, phenotyping, and studying these large populations are substantial, recently estimated at three billion dollars for 500,000 individuals. They are also decade-long efforts. We hypothesized that automation and analytic tools can repurpose the informational byproducts of routine clinical care, bringing sample acquisition and phenotyping to the same high-throughput pace and commodity price-point as is currently true of genome-wide genotyping. Described here is a demonstration of the capability to acquire samples and data from densely phenotyped and genotyped individuals in the tens of thousands fo...

Possible roles of microglial cells for neurotoxicity in clinical neurodegenerative diseases and experimental animal models.

Inflammation and Allergy Drug Targets — Mon, 31 Aug 2009 23:00:00 +0100

Authors: Sugama S, Takenouchi T, Cho BP, Joh TH, Hashimoto M, Kitani H Microglia has been demonstrated to play critical roles in various neurodegenerative disorders, such as Parkinson's disease (PD), Alzheimer's disease (AD), Huntington's disease (HD) as well as neuroinflammatory disorders including AIDS encephalitis, multiple sclerosis. In this manuscript, we review the possible roles of microglial cells in animal models of these clinical disorders and human clinical cases. Activated microglia has been demonstrated in various brain regions, such as the hippocampus, substantia nigra and cortex in PD, AD and HD. The contribution of microglial cells to these neurodegenerative disorders is supported by findings in animal experiments: (1) microglial activation precedes the neurodegenerativ...

Epidemiology Seminar Series

DF/HCC: Latest News — Mon, 31 Aug 2009 16:20:00 +0100

Wednesday, September 23, 200912:30 p.m.Speaker: David ChristianiProfessor of Occupational Medicine and Epidemiology; Professor of MedicineHarvard Medical SchoolBoston, MA TITLE: Genetic Predictors of Survival in Lung Cancer12:30 p.m.Kresge Building Room 502677 Huntington AvenueBoston, MassachusettsSponsored by DF/HCC and the Epidemiology Department at HSPH (Source: DF/HCC: Latest News)

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RNAi for the Treatment of Prion Disease: A Window for Intervention in Neurodegeneration?

CNS and Neurological Disorders Drug Targets — Thu, 27 Aug 2009 22:10:05 +0100

Authors: White MD, Mallucci GR Effective treatment of neurodegenerative disease is one of the major challenges facing biomedical research. These disorders, which include Alzheimer's, Huntington's and Parkinson's diseases - as well as the rarer prion diseases - constitute an ever-increasing burden in the developed world, socially, medically and economically. The key barrier to effective therapy is that they present clinically when neuronal loss is advanced, and irreversible. Current treatments are almost all directed at modifying symptoms; few address underlying pathogenic mechanisms and are inevitably delivered too late to rescue dying neurons. In the field of prion diseases, however, insights into the molecular basis and the temporal evolution of prion neurotoxicity are increasing. Re...

BAG1 modulates huntingtin toxicity, aggregation, degradation, and subcellular distribution

Journal of Neurochemistry — Wed, 26 Aug 2009 23:00:00 +0100

Bcl-2-associated athanogene-1 (BAG1) is a multifunctional protein delivering chaperone-recognized unfolded substrates to the proteasome for degradation. It has been shown to be essential for proper CNS development in vivo, playing a crucial role in neuronal survival and differentiation. With regard to Huntington's disease, a sequestration of BAG1 into inclusion bodies and a neuroprotective effect in double transgenic mice have been reported. Here, we show that BAG1 reduces aggregation and accelerates degradation of mutant huntingtin (htt-mut). Moreover, it reduces nuclear levels of htt-mut. This effect can be overcome by over-expression of seven in absentia homolog 1, an E3 ligase negatively regulated by BAG1 and known to be involved in nuclear import of htt-mut. In vivo, BAG1 proved to be...

Oral Presentations.

Clinical Genetics — Wed, 26 Aug 2009 21:50:27 +0100

Authors: PMID: 19703198 [PubMed - as supplied by publisher] (Source: Clinical Genetics)

Pathogenesis.

Clinical Genetics — Wed, 26 Aug 2009 21:50:24 +0100

Authors: PMID: 19703199 [PubMed - as supplied by publisher] (Source: Clinical Genetics)

Pre-Clinical HD.

Clinical Genetics — Wed, 26 Aug 2009 21:50:22 +0100

Authors: PMID: 19703200 [PubMed - as supplied by publisher] (Source: Clinical Genetics)

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Clinical HD.

Clinical Genetics — Wed, 26 Aug 2009 21:50:20 +0100

Authors: PMID: 19703201 [PubMed - as supplied by publisher] (Source: Clinical Genetics)

Medical Treatments.

Clinical Genetics — Wed, 26 Aug 2009 21:50:18 +0100

Authors: PMID: 19703202 [PubMed - as supplied by publisher] (Source: Clinical Genetics)

Animal Models.

Clinical Genetics — Wed, 26 Aug 2009 21:50:16 +0100

Authors: PMID: 19703203 [PubMed - as supplied by publisher] (Source: Clinical Genetics)

Care/Health Services.

Clinical Genetics — Wed, 26 Aug 2009 21:50:14 +0100

Authors: PMID: 19703204 [PubMed - as supplied by publisher] (Source: Clinical Genetics)

Social and Family Issues.

Clinical Genetics — Wed, 26 Aug 2009 21:50:11 +0100

Authors: PMID: 19703205 [PubMed - as supplied by publisher] (Source: Clinical Genetics)

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Epidemiology.

Clinical Genetics — Wed, 26 Aug 2009 21:50:09 +0100

Authors: PMID: 19703206 [PubMed - as supplied by publisher] (Source: Clinical Genetics)

Genetics/Predictive Testing.

Clinical Genetics — Wed, 26 Aug 2009 21:50:07 +0100

Authors: PMID: 19703207 [PubMed - as supplied by publisher] (Source: Clinical Genetics)

Late Breaking Abstracts.

Clinical Genetics — Wed, 26 Aug 2009 21:50:04 +0100

Authors: PMID: 19703208 [PubMed - as supplied by publisher] (Source: Clinical Genetics)

The monkeys with three parents that could help prevent some genetic diseases

Guardian Unlimited Science — Wed, 26 Aug 2009 21:14:49 +0100

• DNA replaced by material from donor egg• Technique could eradicate mitochondrial disordersFour baby monkeys created in a laboratory in the United States could hold the key to the eradication of a class of incurable genetic diseases, scientists revealed today.In an experiment that brings the creation of babies with three biological parents a step closer, Spindler, Spindly and twins Mito and Tracker were born through IVF using a technique that should make it possible to prevent women who carry genetic disorders of the mitochondria from passing them on to their children.Defects in mitochondria – tiny structures known as the power houses or batteries of a cell because they convert food into energy – affect about one in 6,500 births in the UK and can cause about 50 known diseases, suc...

Monkeys with three parents

Guardian Unlimited Science — Wed, 26 Aug 2009 21:14:49 +0100

• DNA replaced by material from donor egg• Technique could eradicate mitochondrial disorders阅读中文 | Read this in ChineseFour baby monkeys created in a laboratory in the United States could hold the key to the eradication of a class of incurable genetic diseases, scientists revealed today.In an experiment that brings the creation of babies with three biological parents a step closer, Spindler, Spindly and twins Mito and Tracker were born through IVF using a technique that should make it possible to prevent women who carry genetic disorders of the mitochondria from passing them on to their children.Defects in mitochondria – tiny structures known as the power houses or batteries of a cell because they convert food into energy – affect about one in 6,500 births in the UK and can...

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AAFP-CIHI09: Parasitology handout (Mark Huntington MD, PhD)

Family Medicine Digital Resources Library (FMDRL) Recently Uploaded — Tue, 25 Aug 2009 23:00:00 +0100

(Source: Family Medicine Digital Resources Library (FMDRL) Recently Uploaded)

The Failure of Mitochondria Leads to Neurodegeneration: Do Mitochondria Need A Jump Start?

Advanced Drug Delivery Reviews — Tue, 25 Aug 2009 23:00:00 +0100

Authors: Lee J, Boo JH, Ryu H Mitochondria are the power engine generating biochemical energy in the cell. Mitochondrial dysfunction and bioenergy deficiency is closely linked to the pathogenesis of neurodegenerative disorders. Mitochondria play a variety of roles by integrating extracellular signals and executing important intracellular events in neuronal survival and death. In this context, the regulation of mitochondrial function via therapeutic approaches may exert some salutary and neuroprotective mechanisms. Understanding the relationship of mitochondria-dependent pathogenesis may provide important pharmacological utility in the treatment of neurodegenerative conditions such as Alzheimer's disease, amyotrophic lateral sclerosis, Huntington's disease and Parkinson's disease. Indee...

Protective effect of hesperidin and naringin against 3-nitropropionic acid induced Huntington's like symptoms in rats: possible role of nitric oxide.

Behavioural Brain Research — Tue, 25 Aug 2009 23:00:00 +0100

Authors: Kumar P, Kumar A 3-nitropropionic acid (3-NP) is a well known experimental model to study Huntington's disease (HD) and associated neuropsychiatric problems. Present study has been designed to explore the protective effects of hesperidin, naringin, and their nitric oxide mechanism (if any) against 3-nitropropionic acid induced neurotoxicity in rats. Systemic 3-nitropropionic acid (10mg/kg) treatment for 14 days in rats significantly induced HD like symptoms in rats as indicated by reduced locomotor activity, body weight, grip strength, oxidative defense and mitochondrial complex enzymes (complex -I, II, and IV) activities in striatum. Naringin and hesperidin pretreatment significantly attenuated behavioural alterations, oxidative stress and mitochondrial enzyme complex dysfunc...

The Epidemiology of Helicobacter pylori and Public Health Implications

Helicobacter — Tue, 25 Aug 2009 12:14:37 +0100

This article presents a review of the literature on the epidemiology and public health implications of Helicobacter pylori infection published from April 2008 through to March 2009. The authors used MeSH terms "Helicobacter infections epidemiology,""Helicobacter infections prevention and control" to search multiple databases (PubMed, Embase, Cochrane, Cochrane Library, EBMR, BIOSIS), and independently searched PubMed using the term "Helicobacter" with "Epidemiology,""Transmission,""Prevalence" or "Environment." Articles without topical relevance were excluded. Two additional papers known to the authors were added. The identified literature is summarized by subtopic: reviews; prevalence; incidence; transmission; risk factors; and public health policy. (Source: Helicobacter)

A stable G-quartet binds to a huntingtin protein fragment containing expanded polyglutamine tracks

Journal of Neuroscience Research — Mon, 24 Aug 2009 23:00:00 +0100

Huntington's disease (HD) is a progressive neurodegenerative disorder that is inherited in an autosomal dominant fashion. The disease is the result of an expanded CAG repeat in exon 1 of the HD gene, which encodes an elongated polyglutamine tract in the mutant form of the protein, huntingtin. Disease pathogenesis is linked to intracellular aggregates that form because of the tendency of the mutant protein to misfold. The role of huntingtin aggregates in disease pathology is unclear; it has been proposed that the aggregates themselves are toxic because of their ability to sequester intracellular proteins and disrupt normal cellular function. In addition, the mechanistic steps that lead to aggregate formation appear to be central to HD pathology. We have previously reported that guanosine-ri...

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[Basal ganglia and psychiatric disorders: An experimental validation.]

Annales Pharmaceutiques Francaises — Mon, 24 Aug 2009 05:18:19 +0100

Authors: Féger J, Worbe Y, Galineau L, Tremblay L Abnormal movements and behavioral disorders are characteristic manifestations observed in certain neuropsychiatric diseases such as Tourette's syndrome or Huntington Disease. Together with brain imaging findings, the clinical data could suggest a relationship with basal ganglia dysfunction. In the first part of this review, we recall the anatomic relationships existing, via segregated cortico-cortical circuits, between these structures and the cortical areas having motor and cognitive or motivational-emotional attributes. This structure suggests that in pathologies like Parkinson's or Huntington disease cognitive and motivational-emotional disorders as well motor disturbances could be related to lesions or dysfunctions involving in...

Emerging Subspecialties in Neurology: Translational research in movement disorders

Neurology — Sun, 23 Aug 2009 23:00:00 +0100

(Source: Neurology)

Uterine compression suture against impending recurrence of uterine inversion immediately after laparotomy repositioning

Journal of Obstetrics and Gynaecology Research — Sun, 23 Aug 2009 23:00:00 +0100

Uterine compression suture to prevent impending recurrence of uterine inversion after laparotomy repositioning has not been reported previously. A 28-year-old primigravid woman demonstrated uterine inversion. Although Huntington's procedure had successfully repositioned the uterus, acute recurrence of inversion with uterine atony appeared to be impending during the surgery. We applied modified uterine compression sutures, which prevented the recurrence of inversion and achieved complete hemostasis. Three years later, the patient became pregnant and vaginally delivered a term infant. Thereafter, uterine inversion recurred, and was repositioned manually. Uterine compression suture may be of use not only for hemostasis for postpartum hemorrhage, but also for prophylaxis against acute recurren...

Huntingtons disease - video

Neurological Conditions Specialist Library — Fri, 21 Aug 2009 17:27:32 +0100

In this patient information video an expert explains the effects of Huntington's disease. Lee, 39, talks about his life with Huntington's and the importance of getting tested if you have a family history of it. (Source: Neurological Conditions Specialist Library)

Viability Of Bone Marrow Stem Cells With Unique MRI Tracking Methods Shown By TAU Researcher

Alzheimer's / Dementia News From Medical News Today — Fri, 21 Aug 2009 07:00:00 +0100

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SC: MRI shows possibilities of bone marrow stem cell therapy

Health Imaging News — Thu, 20 Aug 2009 15:50:27 +0100

In vivo MRI results demonstrate that mesenchymal stem cells migrate toward a quinolinic acid lesion and can survive for 19 days post-transplantation, which gives hope for future research harnessing stem cells for treating neurodegenerative diseases, such as Huntington's, Alzheimer's and Parkinson's, according to research in this month’s issue of Stem Cells. (Source: Health Imaging News)

SC: MRI shows possibilities of bone marrow stem cell therapy

Health Imaging News — Thu, 20 Aug 2009 15:50:27 +0100

Stem Cells Repair the Human Brain

Medicineworld.org: New Article Alert — Thu, 20 Aug 2009 12:43:23 +0100

There is no known cure for neurodegenerative diseases such as Huntington's, Alzheimer's and Parkinson's. But new hope, in the form of stem cells created from the patient's own bone marrow, can be found - and literally seen - in laboratories at Tel Aviv University. Dr. Yoram Cohen of TAU's School of Chemistry has recently proven the viability of these innovative stem cells, called mesenchymal stem cells, using in-vivo MRI. Dr. Cohen has been able to track their progress within the brain, and initial studies indicate they can identify unhealthy or damaged tissues, migrate to them, and potentially repair or halt cell degeneration. His findings have been published in the journal Stem Cells........ (Source: Medicineworld.org: New Article Alert)

Republication: In That Case

Journal of Bioethical Inquiry — Wed, 19 Aug 2009 18:48:37 +0100

Content Type Journal ArticleDOI 10.1007/s11673-009-9185-yAuthors Dan Brock, Harvard Medical School, Harvard University Department of Global Health and Social Medicine, Division of Medical Ethics FXB Building, 651 Huntington Avenue, 6th Floor Boston MA 02115 USA Journal Journal of Bioethical InquiryOnline ISSN 1872-4353Print ISSN 1176-7529 (Source: Journal of Bioethical Inquiry)

Disruption of Rab11 activity in a knock-in mouse model of Huntington's Disease.

Neurobiology of Disease — Tue, 18 Aug 2009 23:00:00 +0100

Authors: Li X, Sapp E, Chase K, Comer-Tierney LA, Masso N, Alexander J, Reeves P, Kegel KB, Valencia A, Esteves M, Aronin N, Difiglia M The Huntington's disease (HD) mutation causes polyglutamine expansion in huntingtin (Htt) and neurodegeneration. Htt interacts with a complex containing Rab11GDP and is involved in activation of Rab11, which functions in endosomal recycling and neurite growth and long-term potentiation. Like other Rab proteins, Rab11GDP undergoes nucleotide exchange to Rab11GTP for its activation. Here we show that striatal membranes of HD(140Q/140Q) knock-in mice are impaired in supporting conversion of Rab11GDP to Rab11GTP. Dominant negative Rab11 expressed in the striatum and cortex of normal mice caused neuropathology and motor dysfunction, suggesting that a defici...

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THE WORLD IN MEDICINE: Huntington Disease Setback

JAMA — Mon, 17 Aug 2009 23:00:00 +0100

(Source: JAMA)

Accumulation of ubiquitin conjugates in a polyglutamine disease model occurs without global ubiquitin/proteasome system impairment [Medical_Sciences]

Proceedings of the National Academy of Sciences — Mon, 17 Aug 2009 23:00:00 +0100

Aggregation-prone proteins have been suggested to overwhelm and impair the ubiquitin/proteasome system (UPS) in polyglutamine (polyQ) disorders, such as Huntington's... (Source: Proceedings of the National Academy of Sciences)

Weight loss in huntington disease increases with higher cag repeat number

Neurology — Sun, 16 Aug 2009 23:00:00 +0100

(Source: Neurology)

Bile acids: regulation of apoptosis by ursodeoxycholic acid [Thematic Reviews]

The Journal of Lipid Research — Sun, 16 Aug 2009 23:00:00 +0100

Bile acids are a group of molecular species of acidic steroids with peculiar physical-chemical and biological characteristics. At high concentrations they become toxic to mammalian cells, and their presence is pertinent in the pathogenesis of several liver diseases and colon cancer. Bile acid cytoxicity has been related to membrane damage, but also to nondetergent effects, such as oxidative stress and apoptosis. Strikingly, hydrophilic ursodeoxycholic acid (UDCA), and its taurine-conjugated form (TUDCA), show profound cytoprotective properties. Indeed, these molecules have been described as potent inhibitors of classic pathways of apoptosis, although their precise mode of action remains to be clarified. UDCA, originally used for cholesterol gallstone dissolution, is currently considered th...

Diminished hippocalcin expression in Huntington's disease brain does not account for increased striatal neuron vulnerability as assessed in primary neurons

Journal of Neurochemistry — Sun, 16 Aug 2009 23:00:00 +0100

Hippocalcin is a neuronal calcium sensor protein previously implicated in regulating neuronal viability and plasticity. Hippocalcin is the most highly expressed neuronal calcium sensor in the medium spiny striatal output neurons that degenerate selectively in Huntington's disease (HD). We have previously shown that decreased hippocalcin expression occurs in parallel with the onset of disease phenotype in mouse models of HD. Here we show by in situ hybridization histochemistry that hippocalcin RNA is also diminished by 63% in human HD brain. These findings lead us to hypothesize that diminished hippocalcin expression might contribute to striatal neurodegeneration in HD. We tested this hypothesis by assessing whether restoration of hippocalcin expression would decrease striatal neurodegenera...

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Synthesis of fluorescent-maghemite nanoparticles as multimodal imaging agents for amyloid-beta fibrils detection and removal by a magnetic field.

Biochemical and Biophysical Research communications — Fri, 14 Aug 2009 07:38:33 +0100

Authors: Skaat H, Margel S Early diagnosis in Alzheimer's disease (AD), before the onset of marked clinical symptoms, is critical in preventing the irreversible neuronal damage that eventually leads to dementia and ultimately death. Therefore, there is an urgent need for in vivo imaging agents, which are valuable as specific biomarkers to demonstrate the location and density of amyloid plaques in the living human brain. The present manuscript describes a novel method for selective marking of Abeta(40) fibrils by non-fluorescent gamma-Fe(2)O(3) and fluorescent-magnetic gamma-Fe(2)O(3)-rhodamine or gamma-Fe(2)O(3)-Congo red nanoparticles, and the complete removal of the magnetized fibrils from the aqueous continuous phase by a magnetic field. These fluorescent-maghemite nanoparticles as ...

RNA Gain-of-Function in Spinocerebellar Ataxia Type 8

PLoS Genetics — Thu, 13 Aug 2009 23:00:00 +0100

We describe several lines of evidence that RNA gain-of-function effects play a significant role in spinocerebellar ataxia type 8 (SCA8) and has broader implications for understanding the CNS effects of other trinucleotide expansion disorders including myotonic dystrophy type 1, Huntington disease like-2, and spinocerebellar ataxia type 7. The SCA8 mutation is bidirectionally transcribed resulting in the expression of CUGexp transcripts from ATXN8OS and CAGexp transcripts and polyglutamine protein from the overlapping ATXN8 gene. These data suggest that SCA8 pathogenesis involves toxic gain-of-function effects at the RNA (CUGexp) and/or protein (PolyQ) levels. We present three lines of evidence that CUGexp transcripts play a significant role in SCA8: 1) CUGexp transcripts accumulate as ribo...

The pathogenic mechanisms of polyglutamine diseases and current therapeutic strategies

Journal of Neurochemistry — Wed, 12 Aug 2009 23:00:00 +0100

Expansion of CAG trinucleotide repeat within the coding region of several genes results in the production of proteins with expanded polyglutamine (PolyQ) stretch. The expression of these pathogenic proteins leads to PolyQ diseases, such as Huntington's disease or several types of spinocerebellar ataxias. This family of neurodegenerative disorders is characterized by constant progression of the symptoms and molecularly, by the accumulation of mutant proteins inside neurons causing their dysfunction and eventually death. So far, no effective therapy actually preventing the physical and/or mental decline has been developed. Experimental therapeutic strategies either target the levels or processing of mutant proteins in an attempt to prevent cellular deterioration, or they are aimed at the dow...

Kaempferol protects against rat striatal degeneration induced by 3-nitropropionic acid.

Brain Research — Wed, 12 Aug 2009 23:00:00 +0100

Authors: Lagoa R, Lopez-Sanchez C, Samhan-Arias AK, Gañan CM, Garcia-Martinez V, Gutierrez-Merino C Abstract 3-Nitropropionic acid (NPA) produces degeneration of striatum and some neurological disturbances characteristic of Huntington's disease in rodents and primates. We have shown that the flavonoid kaempferol largely reduced striatal damage induced by cerebral ischemia-reperfusion in rats (Lopez-Sanchez et al. 2007, Brain Res.1182, 123-137). In this work, we report that intraperitoneal (i.p.) administration of kaempferol affords an efficient protection against NPA-induced neurodegeneration in Wistar rats. We studied the effects of daily i.p. injections of 7, 14 and 21 mg of kaempferol/kg body weight during the NPA-treatment (25 mg/kg body weight/12h i.p., for 5 days) on the neu...

Downstream mechanisms triggered by mitochondrial dysfunction in the basal ganglia: From experimental models to neurodegenerative diseases.

Biochimica et Biophysica Acta — Wed, 12 Aug 2009 23:00:00 +0100

Authors: Gubellini P, Picconi B, Di Filippo M, Calabresi P Mitochondrial dysfunctions have been implicated in the cellular processes underlying several neurodegenerative disorders affecting the basal ganglia. These include Huntington's chorea and Parkinson's disease, two highly debilitating motor disorders for which recent research has also involved gene mutation linked to mitochondrial deficits. Experimental models of basal ganglia diseases have been developed by using toxins able to disrupt mitochondrial function: these molecules act by selectively inhibiting mitochondrial respiratory complexes, uncoupling cellular respiration. This in turn leads to oxidative stress and energy deficit that trigger critical downstream mechanisms, ultimately resulting in neuronal vulnerability and loss...

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Kaempferol protects against rat striatal degeneration induced by 3-nitropropionic acid

Journal of Neurochemistry — Wed, 12 Aug 2009 23:00:00 +0100

3-Nitropropionic acid (NPA) produces degeneration of striatum and some neurological disturbances characteristic of Huntington's disease in rodents and primates. We have shown that the flavonoid kaempferol largely reduced striatal damage induced by cerebral ischaemia-reperfusion in rats (Lopez-Sanchez et al. 2007). In this work, we report that intraperitoneal (i.p.) administration of kaempferol affords an efficient protection against NPA-induced neurodegeneration in Wistar rats. We studied the effects of daily i.p. injections of 7, 14 and 21 mg of kaempferol/kg body weight during the NPA-treatment (25 mg/kg body weight/12 h i.p., for 5 days) on the neurological deficits, degeneration of rat striatum and oxidative stress markers. Intraperitoneal injections of 14[ndash]21 mg of kaempferol/kg ...

I will die the most horrible death

Guardian Unlimited Science — Mon, 10 Aug 2009 23:05:20 +0100

As a reporter for NBC, Charles Sabine had experienced the horrors of war. But that was nothing compared with discovering he had inherited Huntington's diseaseI was travelling with a camera crew and a translator into the Doboj pocket in northern Bosnia in 1995. We stumbled into a clearing in the middle of this wooded hilly area. Mujahideen fighters surrounded our Land Rover and held us in the vehicle while they discussed what to do with us. Meanwhile, a kid came over, smashed his way into the car and held a grenade up to my face. He then pulled the pin. At that moment, a Bosnian army colonel drove up, recognised the translator and calmed the Mujahideen down. He got the boy to put the pin back into the grenade.A terrifying experience – but not as terrifying as getting tested for Huntington...

Clinical and genetic characteristics of Mexican Huntington's disease patients

Movement Disorders — Mon, 10 Aug 2009 23:00:00 +0100

We report the characteristics of 691 Mexican patients with Huntington's disease (HD). These patients, representing 401 families, constitute the largest series of Mexican HD cases as yet described in the literature. We found the clinical characteristics of these patients to be similar to those of other populations, but we observed a higher frequency of infantile cases, a shorter disease duration and a lower suicide rate. In 626 cases, for which molecular analyses were available, CAG-trinucleotide expansion size ranged from 37-106 repeats. The large number of CAG repeats (19.04 ± 3.02) in normal alleles and the presence of new mutations suggest that the overall prevalence of HD in the Mexican population could be expected to be within range of, or higher than, that reported for Europeans. ©...

Mitochondria in Huntington's disease.

Biochimica et Biophysica Acta — Mon, 10 Aug 2009 23:00:00 +0100

Authors: Damiano M, Galvan L, Déglon N, Brouillet E Huntington's disease (HD) is an inherited progressive neurodegenerative disorder associated with involuntary abnormal movements (chorea), cognitive deficits and psychiatric disturbances. The disease is caused by an abnormal expansion of a CAG repeat located in exon 1 of the gene encoding the huntingtin protein (Htt) that confers a toxic function to the protein. The most striking neuropathological change in HD is the preferential loss of medium spiny GABAergic neurons in the striatum. The mechanisms underlying striatal vulnerability in HD are unknown, but compelling evidence suggests that mitochondrial defects may play a central role. Here we review recent findings supporting this hypothesis. Studies investigating the toxic effect...

[Articles] Biological and clinical manifestations of Huntington's disease in the longitudinal TRACK-HD study: cross-sectional analysis of baseline data

Lancet Neurology — Sun, 09 Aug 2009 23:00:00 +0100

Huntington's disease (HD) is an autosomal dominant, fully penetrant, neurodegenerative disease that most commonly affects adults in mid-life. Our aim was to identify sensitive and reliable biomarkers in premanifest carriers of mutated HTT and in individuals with early HD that could provide essential methodology for the assessment of therapeutic interventions. (Source: Lancet Neurology)

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NEUROLOGICAL REVIEW: Progress and Challenges in RNA Interference Therapy for Huntington Disease

Archives of Neurology — Sun, 09 Aug 2009 23:00:00 +0100

Huntington disease is an incurable, dominant neurodegenerative disorder caused by polyglutamine repeat expansion in the huntingtin protein. Reducing mutant huntingtin expression may offer a treatment for Huntington disease. RNA interference has emerged as a powerful method to silence dominant disease genes. As such, it is being developed as a prospective Huntington disease therapy. Here I discuss the current progress and important remaining challenges of RNA interference therapy for Huntington disease. (Source: Archives of Neurology)

A peek inside the Huntington's brain: Will functional imaging take us one step closer in solving the puzzle?

Experimental Neurology — Sun, 09 Aug 2009 23:00:00 +0100

Authors: Georgiou-Karistianis N PMID: 19679124 [PubMed - as supplied by publisher] (Source: Experimental Neurology)

Blood test for mothers could save lives of hundreds of unborn babies

Guardian Unlimited Science — Sun, 09 Aug 2009 18:55:56 +0100

• Risk-free screening may replace amniocentesis test • Diagnosis would use maternal blood sample The NHS is developing a simple blood test that could save the lives of hundreds of unborn babies who are put at risk when doctors try to establish whether they are developing healthily in the womb, the Guardian has learned.The test could put an end to the use of invasive procedures such as amniocentesis, which cause some women to miscarry.The Guardian has been given exclusive access to the NHS's £2m research into a new technology which, if successful, would benefit the thousands of women a year whose babies are identified as being at high risk of being born with a condition such as cystic fibrosis, Down's syndrome or Duchenne muscular dystrophy.The early signs are so promising that the pro...

Blood test could save lives of hundreds of babies

Guardian Unlimited Science — Sun, 09 Aug 2009 18:55:56 +0100

Circulating IL-18 and the risk of type 2 diabetes in women

Diabetologia — Sun, 09 Aug 2009 07:08:49 +0100

Conclusions/interpretation Elevated IL-18 levels are associated with higher risk of diabetes. This association is independent of usual risk factors, including BMI and adipokine levels. Content Type Journal ArticleCategory ArticleDOI 10.1007/s00125-009-1455-zAuthors M. F. Hivert, Massachusetts General Hospital General Medicine Division Boston MA USAQ. Sun, Harvard School of Public Health Departments of Nutrition and Epidemiology 665 Huntington Avenue Boston MA 02115 USAP. Shrader, Massachusetts General Hospital General Medicine Division Boston MA USAC. S. Mantzoros, Beth Israel Deaconess Medical Center and Harvard Medical School Division of Endocrinology, Diabetes and Metabolism Boston MA USAJ. B. Meigs, Massachusetts General Hospital General Medicine Division Boston MA...

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Homodyne locking of a squeezer

Optics Letters — Sat, 08 Aug 2009 12:14:01 +0100

We report on the successful implementation of an approach to locking the frequencies of an optical parametric oscillator (OPO)-based squeezed-vacuum source and its driving laser. The technique allows the simultaneous measurement of the phase shifts induced by a cavity, which may be used for the ... (Source: Optics Letters)

HSP40 Ameliorates Impairment of Insulin Secretion by Inhibiting Huntingtin Aggregation in a HD Pancreatic beta Cell Model.

Bioscience, Biotechnology, and Biochemistry — Thu, 06 Aug 2009 23:00:00 +0100

Authors: Ye CF, Li H Diabetes frequently develops in Huntington's disease patients. Here, we found that mutant huntingtin forms aggregates in the cytoplasm and reduces insulin secretion from huntingtin transfected pancreatic beta cell lines, NIT-1 cells. Activity of the pro-survival factor, Akt, is enhanced in these cells, which might improve the maintenance of insulin content. Overexpression of heat shock protein 40 (HSP40) inhibits aggregation, reverses impaired insulin release, and blocks the enhancement of Akt activity. These results suggest that impairment of beta cells is mostly linked with the aggregate formation of mutant huntingtin, and that HSP40 ameliorates the malfunction of pancreatic beta cells by inhibiting aggregation. PMID: 19661690 [PubMed - as supplied by publish...

Meet katy fetters

Cerebral Palsy International Research Foundation News — Thu, 06 Aug 2009 05:34:31 +0100

Katy Fetters is a seventeen year-old girl who has hemiplegic cerebral palsy. She lives in Orange County, California and attends Huntington Beach High School read more (Source: Cerebral Palsy International Research Foundation News)

Instrumenting the health care enterprise for discovery research in the genomic era [RESOURCES]

Genome Research — Wed, 05 Aug 2009 23:00:00 +0100

Bell's palsy and choreiform movements during peginterferon alpha and ribavirin therapy.

World Journal of Gastroenterology : WJG — Wed, 05 Aug 2009 21:54:11 +0100

We report a case of Bell's palsy in a patient with chronic hepatitis C during peginterferon-alpha and ribavirin therapy. The patient subsequently developed clinically inapparent facial nerve involvement on the contralateral side and showed an increase in choreic movements related to Huntington's disease during treatment. PMID: 19653353 [PubMed - as supplied by publisher] (Source: World Journal of Gastroenterology : WJG)

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Prana is Granted Key Patent by European Patent Office

Medical News (via PRIMEZONE) — Wed, 05 Aug 2009 12:31:00 +0100

NEW YORK, Aug. 5, 2009 (GLOBE NEWSWIRE) -- Prana Biotechnology (Nasdaq:PRAN) (ASX:PBT) today announced that it has secured a key patent protecting its clinical drug asset PBT2, with the European Patent Office issuing a notice of Decision to Grant in Europe. The patent entitled '8-Hydroxyquinoline derivatives' covers the composition of matter of selected families of 8-Hydroxyquinoline compounds, including PBT2 and the uses of such compounds for the treatment of neurological diseases, including Alzheimer's Disease and Huntington's Disease. (Source: Medical News (via PRIMEZONE))

A longitudinal study of economic pressure among people living with a progressive neurological illness.

Chronic Illness — Tue, 04 Aug 2009 23:00:00 +0100

DISCUSSION: The implications of these findings are discussed. They suggest that cut backs in spending, as opposed to income and expenses, are important factors to focus on assisting people to adjust to the changes to their financial situation that frequently occurs after developing one of these progressive neurological illnesses. PMID: 19656811 [PubMed - as supplied by publisher] (Source: Chronic Illness)

Communication and Huntington's disease: qualitative interviews and focus groups with persons with Huntington's disease, family members, and carers.

International Journal of Language and Communication Disorders — Tue, 04 Aug 2009 23:00:00 +0100

Conclusions & Implications: In brief, persons with Huntington's disease expressed a need for a richer social life and more (adjusting) conversation partners, family members expressed a need for more support and professional carers wanted more information about Huntington's disease. The triangular perspective utilized in the present study completed the picture of the communicative consequences of Huntington's disease. In particular, it became clear, that the insights of persons with Huntington's disease can and has to be included in communicative assessments and plans for intervention. PMID: 19657854 [PubMed - as supplied by publisher] (Source: International Journal of Language and Communication Disorders)

Secreted Products from the Porcine Choroid Plexus Accelerate the Healing of.

Cell Transplantation — Tue, 04 Aug 2009 23:00:00 +0100

Authors: Thanos C, Emerich D, Bintz B, Goddard M, Mills J, Jensen R, Lombardi M The choroid plexus (CP), located at the blood-brain interface, is partially responsible for maintaining the composition of cerebrospinal fluid. Epithelial cell clusters isolated from the CP secrete numerous biologically active molecules, and are neuroprotective when transplanted in animal models of Huntington's disease and stroke. The genomic and proteomic profiles of CP may extend beyond CNS applications due to an abundance of trophic and regenerative factors including vascular endothelial growth factor, transforming growth factor beta, and others. Here, we used microarray to investigate the genomic profile of porcine CP epithelium, and then assessed the in vitro and in vivoregenerative capability of secre...

Single-Step Detection of Mutant Huntingtin in Animal and Human Tissues: a BioAssay for Huntington's Disease.

Analytical Biochemistry — Tue, 04 Aug 2009 23:00:00 +0100

We describe a highly sensitive time-resolved FRET assay for quantification of soluble mutant huntingtin in brain, plasma and cerebrospinal fluid. Surprisingly, in mice soluble huntingtin levels decrease during disease progression, inversely correlating with brain aggregate load. Mutant huntingtin is easily detected in human brain and blood-derived fractions predicting a utility to assess mutant huntingtin expression during disease course as well as a pharmacodynamic marker for disease-modifying therapeutics targeting expression, cleavage or degradation of mutant huntingtin. The design of the homogenous one-step method for huntingtin detection is such that it can be easily applied to measure other proteins of interest. PMID: 19664996 [PubMed - as supplied by publisher] (Source: Analytic...

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Single homopolypeptide chains collapse into mechanically rigid conformations [Chemistry]

Proceedings of the National Academy of Sciences — Mon, 03 Aug 2009 23:00:00 +0100

Huntington's disease is linked to the insertion of glutamine (Q) in the protein huntingtin, resulting in polyglutamine (polyQ) expansions that... (Source: Proceedings of the National Academy of Sciences)

Dietary intake in adults at risk for Huntington disease: Analysis of PHAROS Research Participants

Neurology — Sun, 02 Aug 2009 23:00:00 +0100

Conclusions: Increased caloric intake may be necessary to maintain body mass index in clinically unaffected individuals with CAG repeat length ≥37. This may be related to increased energy expenditure due to subtle motor impairment or a hypermetabolic state. (Source: Neurology)

Clinical assessment of mobility and balance impairments in pre-symptomatic Huntington's disease

Gait and Posture — Sun, 02 Aug 2009 23:00:00 +0100

Conclusion: Clinical assessments such as FRT and TUG are not sensitive in detecting motor impairments in individuals with pre-symptomatic HD. The subtle nature of impairments provides a rationale for the use of quantitative gait analysis in pre-symptomatic HD. (Source: Gait and Posture)

What Is Huntington's Disease? What Causes Huntington's Disease?

Health News from Medical News Today — Sun, 02 Aug 2009 07:00:00 +0100

Huntington's disease is an incurable, hereditary brain disorder. It is a devastating brain disorder for which there is no currently 'effective' treatment. Nerve cells become damaged, causing various parts of the brain to deteriorate. (Source: Health News from Medical News Today)