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Orexins Excite Neurons of the Rat Cerebellar Nucleus Interpositus Via Orexin 2 Receptors In Vitro.Email this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
In this study, perfusing slices with orexin A (100 nM-1 muM) or orexin B (100 nM-1 muM) both produced neurons in the rat cerebellar interpositus nucleus (IN) a concentration-dependent excitatory response (96/143, 67.1%). Furthermore, both of the excitations induced by orexin A and B were not blocked by the low-Ca(2+)/high-Mg(2+) medium (n = 8), supporting a direct postsynaptic action of the peptides. Highly selective orexin 1 receptor antagonist SB-334867 did not block the excitatory response of cerebellar IN neurons to orexins (n = 22), but [Ala(11), D-Leu(15)] orexin B, a highly selective orexin 2 receptor (OX(2)R) agoni...
Source: Cerebellum - November 19, 2009 Category: Neuroscience Authors: Yu L, Zhang XY, Zhang J, Zhu JN, Wang JJ Tags: Cerebellum Source Type: journals

Recent Developments in the Understanding of Astrocyte Function in the Cerebellum In Vivo.Email this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
Several studies have contributed to our understanding of astrocytes, especially Bergmann glia, in the cerebellum; but, until recently, none has looked at their function in vivo. Multicell bolus loading of fluorescent calcium indicators in combination with the astrocytic marker SR101 has allowed imaging of up to hundreds of astrocytes at once in the intact cerebellum. In addition, the selective targeting of astrocytes with fluorescent calcium indicator proteins has enabled the study of their function in vivo without the confounding effects of other neuropil signals and with a resolution that surpasses multicell bolus lo...
Source: Cerebellum - November 11, 2009 Category: Neuroscience Authors: Hoogland TM, Kuhn B Tags: Cerebellum Source Type: journals

The Fate of Spontaneous Synchronous Rhythms on the Cerebrocerebellar Loop.Email this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
How does the cerebellum participate in neocortical rhythms? Neocortical signals destined for the cerebellum are integrated in the pontine nuclei (PN) with cerebellar output signals via a direct, reciprocal feedback loop with the cerebellar nuclei (CN). The present study investigated the fate of two spontaneously occurring rhythms in rat neocortex under ketamine anesthesia-slow wave activity at around 1 Hz and gamma oscillations-within this pontonuclear feedback loop. Coordinated oscillatory neuronal activity was studied using simultaneous multineuron recordings in primary motor cortex (M1), PN, and lateral CN. It was r...
Source: Cerebellum - November 10, 2009 Category: Neuroscience Authors: Schwarz C Tags: Cerebellum Source Type: journals

In Vivo Structural and Functional Imaging of the Human Rubral and Inferior Olivary Nuclei: A Mini-review.Email this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
Few imaging studies have been devoted to the structural and functional connectivity of the red and inferior olivary nuclei although these two nuclei represent two main targets of the cerebellum within the brainstem. However, the RN is anatomically and functionally related to a widespread sensorimotor, limbic, and executive brain network. It projects massively onto the principal olive with which it contributes to a cerebello-rubro-olivo-cerebellar loop modulated by cortical and subcortical afferents. Despite a minor role in planning and execution of rhythmic movements, the red nucleus in conjunction with the inferior ol...
Source: Cerebellum - November 7, 2009 Category: Neuroscience Authors: Habas C, Guillevin R, Abanou A Tags: Cerebellum Source Type: journals

Extracerebellar MRI-Lesions in Ataxia Telangiectasia Go Along with Deficiency of the GH/IGF-1 Axis, Markedly Reduced Body Weight, High Ataxia Scores and Advanced Age.Email this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
Ataxia telangiectasia (AT) is a rare autosomal recessive disorder characterized by progressive ataxia, neurodegeneration, immunodeficiency, and cancer predisposition. Pathoanatomical studies reported a degeneration of cerebellar Purkinje cells as the striking feature of the disease. Although recent studies suggested the involvement of extracerebellar structures such as the brainstem and basal ganglia, this has rarely been studied in human AT. Thus, we performed a detailed cliniconeuroradiological investigation of 11 AT patients, aged 8 to 26 years by collecting clinical neurological data, ataxia scores, growth status, ...
Source: Cerebellum - November 7, 2009 Category: Neuroscience Authors: Kieslich M, Hoche F, Reichenbach J, Weidauer S, Porto L, Vlaho S, Schubert R, Zielen S Tags: Cerebellum Source Type: journals

Cellular and Molecular Pathways Triggering Neurodegeneration in the Spinocerebellar Ataxias.Email this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
The autosomal dominant spinocerebellar ataxias (SCAs) are a group of progressive neurodegenerative diseases characterised by loss of balance and motor coordination due to the primary dysfunction of the cerebellum. To date, more than 30 genes have been identified triggering the well-described clinical and pathological phenotype, but the underlying cellular and molecular events are still poorly understood. Studies of the functions of the proteins implicated in SCAs and the corresponding altered cellular pathways point to major aetiological roles for defects in transcriptional regulation, protein aggregation and clearance...
Source: Cerebellum - November 5, 2009 Category: Neuroscience Authors: Matilla-Dueñas A, Sánchez I, Corral-Juan M, Dávalos A, Alvarez R, Latorre P Tags: Cerebellum Source Type: journals

Cerebellar Lingula Size and Experiential Risk Factors Associated with High Levels of Alcohol and Drug Use in Young Adults.Email this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
In conclusion, physical maltreatment was observed to interact with cerebellar morphology resulting in a strong association with alcohol and substance use. Lingula thickness may represent a novel, experientially sensitive, phenotypic risk factor for enhanced alcohol and drug use that perhaps modulates sensitivity to these agents. PMID: 19859774 [PubMed - as supplied by publisher] (Source: Cerebellum)
Source: Cerebellum - October 28, 2009 Category: Neuroscience Authors: Anderson CM, Rabi K, Lukas SE, Teicher MH Tags: Cerebellum Source Type: journals

Cerebellar Disorders-At the Crossroad of Molecular Pathways and Diagnosis.Email this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
Our understanding of the pathogenesis of cerebellar ataxias has started several decades ago and is continuously growing. The numerous mechanisms of cerebellar dysfunction are being discovered by numerous groups of researchers worldwide. Neuronal damage results from a complex interaction of metabolic pathways, which leads to symptoms observed in cerebellar disorders. The main mechanisms at the molecular level are the following: impairment of DNA repair and replication, deregulation of transcription/deficits of processing/transport of RNA, abnormal protein transport and misfolding, aggregates both at the nuclear and cyto...
Source: Cerebellum - October 27, 2009 Category: Neuroscience Authors: Manto M, Marmolino D Tags: Cerebellum Source Type: journals

Predictive Motor Timing Performance Dissociates Between Early Diseases of the Cerebellum and Parkinson's Disease.Email this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
There is evidence that both the basal ganglia and the cerebellum play a role in the neural representation of time in a variety of behaviours, but whether one of them is more important is not yet clear. To address this question in the context of predictive motor timing, we tested patients with various movement disorders implicating these two structures in a motor-timing task. Specifically, we investigated four different groups: (1) patients with early Parkinson's disease (PD); (2) patients with sporadic spinocerebellar ataxia (SCA); (3) patients with familial essential tremor (ET); and (4) matched healthy controls. We u...
Source: Cerebellum - October 23, 2009 Category: Neuroscience Authors: Bareš M, Lungu OV, Husárová I, Gescheidt T Tags: Cerebellum Source Type: journals

Synaptic Inhibition, Excitation, and Plasticity in Neurons of the Cerebellar Nuclei.Email this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
Neurons of the cerebellar nuclei generate the non-vestibular output of the cerebellum. Like other neurons, they integrate excitatory and inhibitory synaptic inputs and filter them through their intrinsic properties to produce patterns of action potential output. The synaptic and intrinsic features of cerebellar nuclear cells are unusual in several respects, however: these neurons receive an overwhelming amount of basal and driven inhibition from Purkinje neurons, but are also spontaneously active, producing action potentials even without excitation. Moreover, not only is spiking by nuclear cells sensitive to the amount...
Source: Cerebellum - October 21, 2009 Category: Neuroscience Authors: Zheng N, Raman IM Tags: Cerebellum Source Type: journals

Commentary of "Brown Gould B, Graybiel AM. Afferents to the Cerebellar Cortex in the Cat: Evidence for an Intrinsic Pathway Leading from the Deep Nuclei to the Cortex. Brain Research 1976;110:601-611"Email this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
PMID: 19841996 [PubMed - as supplied by publisher] (Source: Cerebellum)
Source: Cerebellum - October 19, 2009 Category: Neuroscience Authors: Haines DE, Manto MU, Brown BL, Graybiel A Tags: Cerebellum Source Type: journals

Perinatal Copper Deficiency Alters Rat Cerebellar Purkinje Cell Size and Distribution.Email this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
Copper is required for activity of several key enzymes and for optimal mammalian development, especially within the central nervous system. Copper-deficient (CuD) animals are visibly ataxic, and previous studies in rats have demonstrated impaired motor function through behavioral experiments consistent with altered cerebellar development. Perinatal copper deficiency was produced in Holtzman rat dams by restricting dietary copper during the last two thirds of gestation and lactation. Male offspring were evaluated at postnatal day 25. Compared to cerebella from copper-adequate pups, the CuD pups had larger Purkinje cell ...
Source: Cerebellum - October 16, 2009 Category: Neuroscience Authors: Lyons JA, Prohaska JR Tags: Cerebellum Source Type: journals

GlyT2+ Neurons in the Lateral Cerebellar Nucleus.Email this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
The deep cerebellar nuclei (DCN) are a major hub in the cerebellar circuitry but the functional classification of their neurons is incomplete. We have previously characterized three cell groups in the lateral cerebellar nucleus: large non-GABAergic neurons and two groups of smaller neurons, one of which express green fluorescence protein (GFP) in a GAD67/GFP mouse line and is therefore GABAergic. However, as a substantial number of glycinergic and glycine/GABA co-expressing neurons have been described in the DCN, this classification needed to be refined by considering glycinergic neurons. To this end we took advantage ...
Source: Cerebellum - October 13, 2009 Category: Neuroscience Authors: Uusisaari M, Knöpfel T Tags: Cerebellum Source Type: journals

Chiari II Malformation Mimicking Partial Rhombencephalosynapsis? A Case Report.Email this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
We present the case of a patient with Chiari II malformation mimicking partial rhombencephalosynapsis. PMID: 19821003 [PubMed - as supplied by publisher] (Source: Cerebellum)
Source: Cerebellum - October 9, 2009 Category: Neuroscience Authors: Guntur Ramkumar P, Kanodia AK, Ananthakrishnan G, Roberts R Tags: Cerebellum Source Type: journals

Cerebellar Damage Loosens the Strategic Use of the Spatial Structure of the Search Space.Email this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
The influence of a hemicerebellar lesion on the exploration of environments with different spatial distributions of multiple rewards was analyzed. Hemicerebellectomized (HCbed) and intact rats were submitted to a search task in which they had to explore nine food trays in an open field, avoiding repeated visits. Trays were spatially arranged in four configurations: cross, 3 x 3 matrix, circle, and three clusters of three trays each. Lesioned and intact rats' performances improved in all configurations used. However, the explorative activity of the HCbed animals differed from that of intact rats. Lesioned animals spent ...
Source: Cerebellum - October 1, 2009 Category: Neuroscience Authors: Foti F, Mandolesi L, Cutuli D, Laricchiuta D, De Bartolo P, Gelfo F, Petrosini L Tags: Cerebellum Source Type: journals

Aberrant Splicing of the Senataxin Gene in a Patient with Ataxia with Oculomotor Apraxia Type 2.Email this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
Ataxia with oculomotor apraxia type 2 (AOA2) is caused by a diversity of mutations within the coding region of the senataxin gene. Recently, rare noncoding senataxin mutations affecting RNA processing have been identified in AOA2. Here, we report the case of an 18-year-old woman, with classic clinical features of AOA2, who was found to harbor a mutation within senataxin intron 16. This mutation disrupts the local 5' splice site architecture via a novel intronic frameshift mechanism, causing skipping of exon 16 with predicted disruption of the conserved DNA/RNA helicase domain. RNA processing mutations expand the growin...
Source: Cerebellum - September 1, 2009 Category: Neuroscience Authors: Fogel BL, Lee JY, Perlman S Tags: Cerebellum Source Type: journals

Paraneoplastic Cerebellar Degeneration Mimicking Acute Post-Infectious Cerebellitis.Email this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
Acute cerebellitis is a monophasic non-progressive encephalitis restricted to the cerebellum, while paraneoplastic cerebellar degeneration is a subacute progressive disorder, which may either accompany or herald Hodgkin's disease. In the present report, we describe a young man with clinical and laboratory features of acute post-infectious cerebellitis in whom the progressive relapsing course subsequently led to the diagnosis of Hodgkin's disease. Dynamic changes observed on repeated MRIs during the protracted clinical course imply the presence of early active inflammation and subsequent neuronal atrophy. PMID: 1972...
Source: Cerebellum - September 1, 2009 Category: Neuroscience Authors: Karmon Y, Inbar E, Cordoba M, Gadoth N Tags: Cerebellum Source Type: journals

Increased Concentrations of Nerve Growth Factor and Brain-Derived Neurotrophic Factor in the Rat Cerebellum After Exposure to Environmental Enrichment.Email this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
The molecular mechanism of environmental enrichment (EE) on brain function and anatomy has been partially attributed to the up-regulation of proteins involved in neuronal survival and activity-dependent plasticity, such as the neurotrophins nerve growth factor (NGF) and brain-derived neurotrophic factor (BDNF), in the cerebral cortex and hippocampus of animal models. Nevertheless, at present, little indication is available on the influence of EE on neurotrophin levels in the cerebellum. Thus, in this study, we exposed male Wistar rats to EE from weaning to 5 months of age and evaluated the production of NGF and BDNF in...
Source: Cerebellum - August 18, 2009 Category: Neuroscience Authors: Angelucci F, De Bartolo P, Gelfo F, Foti F, Cutuli D, Bossù P, Caltagirone C, Petrosini L Tags: Cerebellum Source Type: journals

Animal Models of Human Cerebellar Ataxias: a Cornerstone for the Therapies of the Twenty-First Century.Email this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
Cerebellar ataxias represent a group of disabling neurological disorders. Our understanding of the pathogenesis of cerebellar ataxias is continuously expanding. A considerable number of laboratory animals with neurological mutations have been reported and numerous relevant animal models mimicking the phenotype of cerebellar ataxias are becoming available. These models greatly help dissecting the numerous mechanisms of cerebellar dysfunction, a major step for the assessment of therapeutics targeting a given deleterious pathway and for the screening of old or newly synthesized chemical compounds. Nevertheless, difference...
Source: Cerebellum - August 7, 2009 Category: Neuroscience Authors: Manto M, Marmolino D Tags: Cerebellum Source Type: journals

Bergmann Glial S100B Activates Myo-inositol Monophosphatase 1 and Co-localizes to Purkinje Cell Vacuoles in SCA1 Transgenic Mice.Email this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
We examined the expression levels (by Western blotting) of microtubule-associated protein light chain 3 (LC3)-I and LC3-II, and the degradation levels of p62 (a LC3 partner) in the cerebellar fractions prepared from pre-symptomatic SCA1 and age-matched wild-type mice. No p62 degradation was observed; however, LC3-II/(LC3-I + LC3-II) ratios were significantly altered in SCA1 mice indicating changes in the autophagic flux. In addition, LC3 localized to PC vacuoles. Further, we observed a co-localization of myo-inositol monophosphatase 1 (IMPA1) with S100B in PC vacuoles. IMPA1 is present in PC spines and has been implicated ...
Source: Cerebellum - July 10, 2009 Category: Neuroscience Authors: Vig PJ, Shao Q, Subramony SH, Lopez ME, Safaya E Tags: Cerebellum Source Type: journals

Persistent Adeno-associated Virus 2 and Parvovirus B19 Sequences in Post-mortem Human Cerebellum.Email this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
We previously reported in a large cohort (N = 104) of post-mortem tissues the detection of both the non-pathogenic adeno-associated virus (AAV2) in approximately 13% and the pathogenic human parvovirus B19 (B19) in approximately 42% of human brains, particularly the dorsolateral prefrontal cortex. Multiple animal parvoviruses target the developing cerebellum (CBLM) resulting in hypoplasia and ataxia, but very little is known about the human parvoviruses and their ability to infect or cause disease in the CBLM. We have now confirmed in the above cohort the presence of AAV2 and B19 sequences in the CBLM. Our results show...
Source: Cerebellum - July 7, 2009 Category: Neuroscience Authors: Grant JK, Yin NC, Zaytoun AM, Waseem H, Hobbs JA Tags: Cerebellum Source Type: journals

Cerebellar Brain-Derived Neurotrophic Factor, Nerve Growth Factor, and Neurotrophin-3 Expression in Male and Female Rats Is Differentially Affected by Hypergravity Exposure During Discrete Developmental Periods.Email this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
We previously reported that the effects of perinatal exposure to hypergravity on cerebellum and motor functions in rat neonates are strongly dependent on the specific developmental period of exposure. In the present study, we explored the hypothesis that neurodevelopmental changes are associated with altered expression of brain neurotrophins critical for normal brain growth and differentiation. We compared the effects of hypergravity exposure during four developmental periods: period I extended from gestational day (G) 8 through G15; period II from G15 to birth, period III from birth to postnatal day (P) 6; and period ...
Source: Cerebellum - July 2, 2009 Category: Neuroscience Authors: Sajdel-Sulkowska EM, Xu M, Koibuchi N Tags: Cerebellum Source Type: journals

The Treasury of the Commons: Making Use of Public Gene Expression Resources to Better Characterize the Molecular Diversity of Inhibitory Interneurons in the Cerebellar Cortex.Email this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
We mined the Allen Mouse Brain Atlas for genes expressed in cerebellar cortical inhibitory interneurons that would allow identification and possibly distinction of these cells. We identified some 90 genes that are highly expressed in specific subsets of cerebellar cortical inhibitory interneurons or various combinations thereof. Four genes are exclusively expressed, within the cerebellar cortex, in molecular layer interneurons, and ten genes label exclusively inhibitory interneurons in the granule cell layer or subsets thereof. Differential expression of many of these genes in cells residing in the lower versus the upp...
Source: Cerebellum - June 24, 2009 Category: Neuroscience Authors: Schilling K, Oberdick J Tags: Cerebellum Source Type: journals

Topography of Purkinje Cells and Other Calbindin-Immunoreactive Cells Within Adult and Hatchling Turtle Cerebellum.Email this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
The turtle's cerebellum (Cb) is an unfoliated sheet, so the topography of its entire cortex can be easily studied physiologically by optical recordings. However, unlike the mammalian Cb, little is known about the topography of turtle Purkinje cells (PCs). Here, topography was examined using calbindin-D(28K) immunohistochemistry of adult and hatchling turtles (Trachemys scripta elegans, 2.5-15 cm carapace length). Each Cb was flattened between two Sylgard sheets and fixed in paraformaldehyde. Sections (52 microm thick) were cut parallel to the flattened cortex (tangential), resulting in calbindin-immunolabeled PCs being...
Source: Cerebellum - June 22, 2009 Category: Neuroscience Authors: Ariel M, Ward KC, Tolbert DL Tags: Cerebellum Source Type: journals

Role of PACAP in Controlling Granule Cell Migration.Email this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
Pituitary adenylate cyclase-activating polypeptide (PACAP), a neuropeptide, regulates a wide variety of cellular functions, but little is known about its role in neuronal cell migration. Recent studies revealed that PACAP has short-term, cortical layer-specific effects on neuronal cell migration. In this article, we focus on the role of PACAP in controlling the migration of cerebellar granule cells. PMID: 19548046 [PubMed - as supplied by publisher] (Source: Cerebellum)
Source: Cerebellum - June 22, 2009 Category: Neuroscience Authors: Cameron DB, Raoult E, Galas L, Jiang Y, Lee K, Hu T, Vaudry D, Komuro H Tags: Cerebellum Source Type: journals

A Rare Variant of Persistent Trigeminal Artery: Cavernous Carotid-Cerebellar Artery Anastomosis-A Case Report and a Systematic Review.Email this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
We report a very rare anomalous anatomic variant of the cavernous internal carotid artery supplying directly the posterior inferior cerebellar artery, with no basilar artery opacification. A systematic review as well as a description of other variants of trigeminal-cerebellar anastomosis is given. PMID: 19517204 [PubMed - as supplied by publisher] (Source: Cerebellum)
Source: Cerebellum - June 10, 2009 Category: Neuroscience Authors: Raphaeli G, Bandeira A, Mine B, Brisbois D, Lubicz B Tags: Cerebellum Source Type: journals

Functional Imaging of the Deep Cerebellar Nuclei: A Review.Email this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
The present mini-review focused on functional imaging of human deep cerebellar nuclei, mainly the dentate nucleus. Although these nuclei represent the unique output channel of the cerebellum, few data are available concerning their functional role. However, the dentate nucleus has been shown to participate in a widespread functional network including sensorimotor and associative cortices, striatum, hypothalamus, and thalamus, and plays a minor role in motor execution and a major role in sensorimotor coordination and learning, and cognition. The dentate nucleus appears to be predominantly involved in conjunction with th...
Source: Cerebellum - June 9, 2009 Category: Neuroscience Authors: Habas C Tags: Cerebellum Source Type: journals

Computational Models of Timing Mechanisms in the Cerebellar Granular Layer.Email this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
This article will review these computational models and discuss the possible computational power of the cerebellum. PMID: 19495900 [PubMed - as supplied by publisher] (Source: Cerebellum)
Source: Cerebellum - June 4, 2009 Category: Neuroscience Authors: Yamazaki T, Tanaka S Tags: Cerebellum Source Type: journals

Models of Traumatic Cerebellar Injury.Email this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
Traumatic brain injury (TBI) is a major cause of morbidity and mortality worldwide. Studies of human TBI demonstrate that the cerebellum is sometimes affected even when the initial mechanical insult is directed to the cerebral cortex. Some of the components of TBI, including ataxia, postural instability, tremor, impairments in balance and fine motor skills, and even cognitive deficits, may be attributed in part to cerebellar damage. Animal models of TBI have begun to explore the vulnerability of the cerebellum. In this paper, we review the clinical presentation, pathogenesis, and putative mechanisms underlying cerebell...
Source: Cerebellum - June 4, 2009 Category: Neuroscience Authors: Potts MB, Adwanikar H, Noble-Haeusslein LJ Tags: Cerebellum Source Type: journals

PTPRR, cerebellum, and motor coordination.Email this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
Tyrosine phosphorylation is a powerful mechanism of modulation for proliferation, differentiation, and functioning of neurons. The protein products of the neuronal mouse gene PTPRR are physiological regulators of mitogen-activated protein kinase (MAPK) activities. PTPRR(-/-) mice display deficits of motor coordination and balance skills. PTPRR gene orthologues are found in many vertebrates. Recent observations suggest that the human episodic ataxia 2 (EA2) and spinocerebellar ataxia types 6 (SCA6), 12 (SCA12), and 14 (SCA14) might be associated with impaired phosphorylation levels of cerebellum calcium channels and rec...
Source: Cerebellum - May 31, 2009 Category: Neuroscience Authors: Schmitt I, Bitoun E, Manto M Tags: Cerebellum Source Type: journals

The Ataxic Cacna1a-Mutant Mouse Rolling Nagoya: An Overview of Neuromorphological and Electrophysiological Findings.Email this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
Homozygous rolling Nagoya natural mutant mice display a severe ataxic gait and frequently roll over to their side or back. The causative mutation resides in the Cacna1a gene, encoding the pore-forming alpha(1) subunit of Ca(v)2.1 type voltage-gated Ca(2+) channels. These channels are crucially involved in neuronal Ca(2+) signaling and in neurotransmitter release at many central synapses and, in the periphery, at the neuromuscular junction. We here review the behavioral, histological, biochemical, and neurophysiological studies on this mouse mutant and discuss its usefulness as a model of human neurological diseases ass...
Source: Cerebellum - May 29, 2009 Category: Neuroscience Authors: Plomp JJ, van den Maagdenberg AM, Kaja S Tags: Cerebellum Source Type: journals

Cerebellar Contributions to the Processing of Saccadic Errors.Email this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
Saccades are fast eye movements that direct the point of regard to a target in the visual field. Repeated post-saccadic visual errors can induce modifications of the amplitude of these saccades, a process known as saccadic adaptation. Two experiments using the same paradigm were performed to study the involvement of the cerebrum and the cerebellum in the processing of saccadic errors using functional magnetic resonance imaging and in-scanner eye movement recordings. In the first active condition, saccadic adaptation was prevented using a condition in which the saccadic target was shifted to a variable position during t...
Source: Cerebellum - May 26, 2009 Category: Neuroscience Authors: van Broekhoven PC, Schraa-Tam CK, van der Lugt A, Smits M, Frens MA, van der Geest JN Tags: Cerebellum Source Type: journals

Cerebellar Hemorrhage in a Patient with Propionic Acidemia.Email this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
Cerebellar hemorrhage (CH) is a well-known complication in newborns. Among metabolic patients, it has been classically described but rarely reported. This is the first description of a patient with propionic acidemia in whom magnetic resonance imaging (MRI) allowed diagnosis of asymptomatic CH. Due to the usual silent presentation of CH at early ages, we suggest the possibility of including a brain MRI study as part of the routine neurological evaluation in metabolic patients, especially when neurological signs appear. PMID: 19468795 [PubMed - as supplied by publisher] (Source: Cerebellum)
Source: Cerebellum - May 25, 2009 Category: Neuroscience Authors: Velasco-Sánchez D, Gómez-Lopez L, Vilaseca MA, Serrano M, Massaguer S, Campistol J, García-Cazorla A Tags: Cerebellum Source Type: journals

The Ataxic Syrian Hamster: An Animal Model Homologous to the pcd Mutant Mouse?Email this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
A spontaneous model of cerebellar ataxia in the Syrian hamster is described. Breeding data indicate that the condition is hereditary and that the mode of inheritance is autosomal recessive. Homozygotes are smaller in size than the wild-type but have a normal appearance. Mutants show a moderate ataxia beginning at 7 weeks of age. Although affected adults exhibit significant atrophy in the cerebellum, other parts of the brain appear relatively normal by light microscopy. Mutants lose almost all Purkinje cells by 18 months of age and exhibit a moderate reduction in granule cell density, probably as a consequence of the pr...
Source: Cerebellum - May 21, 2009 Category: Neuroscience Authors: Akita K, Arai S Tags: Cerebellum Source Type: journals

State Estimation, Response Prediction, and Cerebellar Sensory Processing for Behavioral Control.Email this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
State estimation has emerged as the keystone in determining the cerebellar contribution to motor control. Clinical, experimental, and neuroimaging data converge to focus on cerebellar multisensory information processing as the mechanism allowing to correctly establish the body state needed for movement. Further, recent data indicate the cerebellum specifically involved in predicting body state changes. The present editorial discusses these evidences and questions cerebellar state estimation in the cognitive domain. PMID: 19455379 [PubMed - as supplied by publisher] (Source: Cerebellum)
Source: Cerebellum - May 19, 2009 Category: Neuroscience Authors: Molinari M, Restuccia D, Leggio MG Tags: Cerebellum Source Type: journals

Balance and Motor Speech Impairment in Essential Tremor.Email this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
The pathogenesis of essential tremor (ET) is still under debate. Several lines of evidence indicate that ET is associated with cerebellar dysfunction. The aim of the present study was to find corroborating evidence for this claim by investigating balance and speech impairments in patients with ET. In addition, the effect of deep brain stimulation (DBS) on balance and speech function was studied. A group of 25 ET patients including 18 with postural and/or simple kinetic tremor (ETpt) and seven ET patients with additional clinical signs of cerebellar dysfunction (ETc) was compared to 25 healthy controls. In addition, 12 ...
Source: Cerebellum - May 18, 2009 Category: Neuroscience Authors: Kronenbuerger M, Konczak J, Ziegler W, Buderath P, Frank B, Coenen VA, Kiening K, Reinacher P, Noth J, Timmann D Tags: Cerebellum Source Type: journals

Cerebellum-Related Characteristics of Scn8a-Mutant Mice.Email this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
Among ten sodium channel alpha-subunit genes mapped in human and mouse genomes, the SCN8A gene is primarily expressed in neurons and glia. Mice with two types of Scn8a null mutations-Scn8a ( med ) and Scn8a ( medTg )-live for only 21-24 days, but those with incomplete mutations-Scn8a ( medJ ) and Scn8a ( medJo )-and those with knockout of Scn8a only in cerebellar Purkinje cells live to adult age. We review here previous work on cerebellum and related regions of Scn8a mutant mice and include some newer immunohistochemical and microchemical results. The resurgent sodium current that underlies the repeated firing of Purki...
Source: Cerebellum - May 7, 2009 Category: Neuroscience Authors: Chen K, Godfrey DA, Ilyas O, Xu J, Preston TW Tags: Cerebellum Source Type: journals

Up-Down Asymmetry of Cerebellar Activation During Vertical Pursuit Eye Movements.Email this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
Animal experiments have demonstrated that the vast majority of vertical gaze-velocity Purkinje cells in the cerebellar floccular lobe, whose firing rate is modulated during vertical smooth pursuit eye movements, show a preference for downward pursuit. Here we validate the functional vertical asymmetry of the cerebellar flocculus in humans using functional magnetic resonance imaging by demonstrating a significantly higher activation of the floccular lobe for downward than for upward pursuit. The findings corroborate our recent hypothesis on the pathogenesis of cerebellar downbeat nystagmus. PMID: 19415407 [PubMed - ...
Source: Cerebellum - May 4, 2009 Category: Neuroscience Authors: Glasauer S, Stephan T, Kalla R, Marti S, Straumann D Tags: Cerebellum Source Type: journals

Fenestration of the Double Origin of the Posterior Inferior Cerebellar Artery Associated with a Contralateral Vertebral Artery Dissection.Email this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
We present the first reported case of fenestration of the double origin of the PICA associated with a dissecting aneurysm of the contralateral vertebral artery (VA) that healed spontaneously after medical observation. PMID: 19408063 [PubMed - as supplied by publisher] (Source: Cerebellum)
Source: Cerebellum - April 30, 2009 Category: Neuroscience Authors: Lee SH, Koh JS, Ryu CW, Bang JS Tags: Cerebellum Source Type: journals

MRI Detection of the Cerebellar Syndrome in Creutzfeldt-Jakob Disease.Email this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
This study was undertaken to explain this apparent paradox. Eleven CJD patients with definite cerebellar or brain stem symptoms were selected from a large prospective study, as well as 11 healthy controls matched for age and gender. All subjects participated in a standardized MRI protocol, including SPGR, fluid-attenuated inversion recovery (FLAIR), DWI and diffusion tensor imaging (DTI). All subjects underwent detailed examination by a neurologist blinded to the radiological findings, who predicted the expected site of cerebral abnormalities. MRI showed good sensitivity for the abnormalities predicted in the cortex (80-90...
Source: Cerebellum - April 30, 2009 Category: Neuroscience Authors: Cohen OS, Hoffmann C, Lee H, Chapman J, Fulbright RK, Prohovnik I Tags: Cerebellum Source Type: journals

Remote Cell Death in the Cerebellar System.Email this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
Functional impairment after focal CNS lesion is highly dependent on damage that occurs in regions that are remote but functionally connected to the primary lesion site. This pattern is particularly evident in the cerebellar system, in which functional interactions between the cerebellar cortex, deep cerebellar nuclei, and precerebellar stations are of paramount importance. Diffuse degeneration after development of a focal CNS lesion has been associated with poor outcomes in several pathologies, such as stroke, multiple sclerosis, and brain trauma. A greater understanding of the mechanisms that underlie the spread of de...
Source: Cerebellum - April 21, 2009 Category: Neuroscience Authors: Viscomi MT, Florenzano F, Latini L, Molinari M Tags: Cerebellum Source Type: journals

Increase in Cerebellar Neurotrophin-3 and Oxidative Stress Markers in Autism.Email this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
Autism is a neurodevelopmental disorder characterized by social and language deficits, ritualistic-repetitive behaviors and disturbance in motor functions. Data of imaging, head circumference studies, and Purkinje cell analysis suggest impaired brain growth and development. Both genetic predisposition and environmental triggers have been implicated in the etiology of autism, but the underlying cause remains unknown. Recently, we have reported an increase in 3-nitrotyrosine (3-NT), a marker of oxidative stress damage to proteins in autistic cerebella. In the present study, we further explored oxidative damage in the aut...
Source: Cerebellum - April 8, 2009 Category: Neuroscience Authors: Sajdel-Sulkowska EM, Xu M, Koibuchi N Tags: Cerebellum Source Type: journals

The Robotic Mouse: Understanding the Role of AF4, a Cofactor of Transcriptional Elongation and Chromatin Remodelling, in Purkinje Cell Function.Email this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
Neurological disorders represent a large share of the disease burden worldwide, and the incidence of age-related forms will continue to rise with life expectancy. Gene targeting has been and will remain a valuable approach to the generation of clinically relevant mouse models from which to elucidate the underlying molecular basis. However, as the aetiology of the majority of these conditions is still unknown, a reverse approach based on large-scale random chemical mutagenesis is now being used in an attempt to identify new genes and associated signalling pathways that control neuronal cell death and survival. Here, we ...
Source: Cerebellum - April 1, 2009 Category: Neuroscience Authors: Bitoun E, Davies KE Tags: Cerebellum Source Type: journals

Malformations of the Midbrain and Hindbrain: A Retrospective Study and Review of the Literature.Email this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
We report the results of a retrospective analysis of radiological and clinical findings in 45 cases of midbrain-hindbrain anomalies and review recent advances in embryology and molecular neurogenetics. Among 45 patients with midbrain-hindbrain malformations, 16 cases of molar tooth malformation, 12 of cerebellar hypoplasia, ten of posterior fossa cyst and cerebellar vermian hypoplasia, three of rhombencephalosynapsis, two of Fukuyama congenital muscular dystrophy and two cases of isolated cerebellar dysplasia were identified. Twenty-six patients presented with motor-mental retardation, which was the most common clinical fi...
Source: Cerebellum - April 1, 2009 Category: Neuroscience Authors: Alkan O, Kizilkilic O, Yildirim T Tags: Cerebellum Source Type: journals

Synchrotron X-ray Fluorescence Reveals Abnormal Metal Distributions in Brain and Spinal Cord in Spinocerebellar Ataxia: A Case Report.Email this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
For the first time, synchrotron rapid-scanning X-ray fluorescence (RS-XRF) was used to simultaneously localize and quantify iron, copper, and zinc in spinal cord and brain in a case of spinocerebellar ataxia (SCA). In the normal medulla, a previously undescribed copper enrichment was seen associated with spinocerebellar fibers and amiculum olivae. This region was virtually devoid of all metals in the SCA case. Regions with neuronal loss and gliosis in the cerebellar cortex, inferior olivary, and dentate nuclei and areas showing loss of myelinated fibers were also low in all metals in SCA compared to control. In contras...
Source: Cerebellum - March 24, 2009 Category: Neuroscience Authors: Popescu BF, Robinson CA, Chapman LD, Nichol H Tags: Cerebellum Source Type: journals

Cuprizone Treatment Induces Distinct Demyelination, Astrocytosis, and Microglia Cell Invasion or Proliferation in the Mouse Cerebellum.Email this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
In this study, we used the cuprizone mouse model to investigate cerebellar demyelination in young adult male mice. The myelin status was analyzed by immunohistochemistry for proteolipoprotein and electron microscopy. The expression and presence of oligodendrocyte, astroglial, and microglia markers were supplementary studied. Cuprizone intoxication induced an almost complete demyelination of cerebellar nuclei. Cerebellar cortex regions were not (cortical gray matter) or only marginally (cortical white matter) affected. In addition, the affected areas displayed hypertrophic and hyperplastic astrocytosis accompanied by microg...
Source: Cerebellum - March 4, 2009 Category: Neuroscience Authors: Groebe A, Clarner T, Baumgartner W, Dang J, Beyer C, Kipp M Tags: Cerebellum Source Type: journals

Experience-Dependent Plasticity of Cerebellar Vermis in Basketball Players.Email this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
The cerebellum is involved in the learning and retention of motor skills. Using animal and human models, a number of studies have shown that long-term motor skill training induces structural and functional plasticity in the cerebellum. The aim of this study was to investigate whether macroscopic alteration in the volume of cerebellum occurs in basketball players who had learned complex motor skills and practiced them intensively for a long time. Three-dimensional magnetic resonance imaging volumetry was performed in basketball players (n = 19) and healthy controls (n = 20), and the volumes of cerebellum and vermian lob...
Source: Cerebellum - March 4, 2009 Category: Neuroscience Authors: Park IS, Lee KJ, Han JW, Lee NJ, Lee WT, Park KA, Rhyu IJ Tags: Cerebellum Source Type: journals

The Role of Kv3-type Potassium Channels in Cerebellar Physiology and Behavior.Email this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
Different subunits of the Kv3 subfamily of voltage-gated potassium (Kv) channels (Kv3.1-Kv3.4) are expressed in distinct neuronal subpopulations in the cerebellum. Behavioral phenotypes in Kv3-null mutant mice such as ataxia with prominent hypermetria and heightened alcohol sensitivity are characteristic of cerebellar dysfunction. Here, we review how the unique biophysical properties of Kv3-type potassium channels, fast activation and fast deactivation that enable cerebellar neurons to generate brief action potentials at high frequencies, affect firing patterns and influence cerebellum-mediated behavior. PMID: 1924...
Source: Cerebellum - February 27, 2009 Category: Neuroscience Authors: Joho RH, Hurlock EC Tags: Cerebellum Source Type: journals

Developmentally Regulated Ca(2+)-Dependent Activator Protein for Secretion 2 (CAPS2) is Involved in BDNF Secretion and is Associated with Autism Susceptibility.Email this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
The postnatal development of the cerebellum is accomplished via a series of cytogenetic and morphogenetic events encoded in the genome. To decipher the underlying genetic basis of these events we have systematized the spatio-temporal gene expression profiles during mouse cerebellar development in the Cerebellar Development Transcriptome Database (CDT-DB). Using the CDT-DB, Ca(2+)-dependent activator protein for secretion 2 (CAPS2 or CADPS2) was identified as a developmentally regulated gene that is predominantly expressed in cerebellar granule cells (GCs) with an expression peak around the first or second postnatal wee...
Source: Cerebellum - February 24, 2009 Category: Neuroscience Authors: Sadakata T, Furuichi T Tags: Cerebellum Source Type: journals

Physiological Purkinje Cell Death Is Spatiotemporally Organized in the Developing Mouse Cerebellum.Email this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
Physiological cell death is crucial for matching defined cellular populations within the central nervous system. Whereas the time course of developmental cell death in the central nervous system is well analyzed, information about its precise spatial patterning is scarce. Yet, the latter one is needed to appraise its contribution to circuit formation and refinement. Here, we document that during normal cerebellar development, dying Purkinje cells were highly localized within the vermal midline and in a lobule specific, parasagittal pattern along the whole mediolateral axis. In addition, single hot spots of cell death l...
Source: Cerebellum - February 24, 2009 Category: Neuroscience Authors: Jankowski J, Miething A, Schilling K, Baader SL Tags: Cerebellum Source Type: journals