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Complex formation between human prostate-specific antigen and protease inhibitors in mouse plasmaEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
When secreted from the prostate, most of prostate-specific antigen (PSA) is free and enzymatically active. Upon reaching circulation, active PSA is inactivated by complex formation with protease inhibitors. To justify the use of mouse models for evaluation of the function of PSA and for studies on therapeutic modalities based on modulation of PSA activity, it is important to know whether PSA complexation is similar in mouse and man.To characterize the circulating forms of PSA in mouse, we used subcutaneous LNCaP and 22RV1 human prostate cancer cell xenograft tumor models. We also added PSA directly to mouse serum. Free and...
Source: The Prostate - November 21, 2009 Category: Urology & Nephrology Authors: Can Hekim, Tero Riipi, Lei Zhu, Pirjo Laakkonen, Ulf-Håkan Stenman, Hannu Koistinen Source Type: journals

Is Milk Thistle OK for Children?Email this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
My son, age three, has been diagnosed with alpha-1 antitrypsin deficiency. His liver enzymes are elevated, but he is in good health. I am very interested in milk thistle for liver support, but his specialist does not agree. Is milk thistle safe for children as a long term supplement? (Source: Dr. Weil Q&A)
Source: Dr. Weil Q&A - November 17, 2009 Category: Consumer Health Advice Source Type: info

Progression of Emphysema Evaluated by MRI Using Hyperpolarized (3)He (HP (3)He) Measurements in Patients with Alpha-1-Antitrypsin (A1AT) Deficiency Compared with CT and Lung Function Tests.Email this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
Conclusion: This pilot study indicates the possible use of nonionizing HP (3)He MRI for monitoring the progression of emphysema. However, in the future, larger studies are needed to confirm these preliminary results. PMID: 19863412 [PubMed - in process] (Source: Acta Radiologica)
Source: Acta Radiologica - November 1, 2009 Category: Radiology Authors: Stavngaard T, Søgaard LV, Batz M, Schreiber LM, Dirksen A Tags: Acta Radiol Source Type: journals

CT scan appearance, densitometry, and health status in protease inhibitor SZ alpha1-antitrypsin deficiency.Email this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
CONCLUSIONS: Subjects with PiSZ showed less emphysema on CT scans, more apical predominance, less abnormal respiratory physiology test results, but similar health status impairment compared with matched subjects with PiZZ. PMID: 19892672 [PubMed - in process] (Source: Chest)
Source: Chest - November 1, 2009 Category: Respiratory Medicine Authors: Holme J, Stockley RA Tags: Chest Source Type: journals

Combination of alpha-1 antitrypsin and doxycycline suppresses collagen-induced arthritisEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
Rheumatoid arthritis (RA) is a complex disease characterized by autoimmune inflammation and joint destruction. Despite recent advances in RA treatment, current therapies require further improvement to overcome adverse events and ineffectiveness in some cases. By targeting different pathways/molecules using drug combinations, a better treatment can be obtained, whereas adverse events are reduced. In order to develop a new treatment option, the present study employs a gene therapy-based combination therapy using doxycycline and human alpha-1 antitrypsin (hAAT).DBA/1 mice were immunized with type II collagen to induce arthrit...
Source: The Journal of Gene Medicine - October 28, 2009 Category: Genetics & Stem Cells Authors: Christian Grimstein, Young-Kook Choi, Minoru Satoh, Yuanqing Lu, Xueying Wang, Martha Campbell-Thompson, Sihong Song Source Type: journals

Hepatoid carcinoma of the ovary with sex cord stromal tumor: a previously unrecognized associationEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
Conclusion  The occurrence of this unreported association of HC with Sertoli-like tumor, the problems of differential diagnosis and therapeutic management of these tumors are the subject of this presentation. A diagnosis of ovarian metastasis from hepatocellular carcinoma is easy in patients with known primary tumor of liver and should be always excluded in these cases as an hepatoid variant of yolk sac tumor. Immunohistochemistry is not useful in these cases. However, a combination of clinical and pathological features is necessary for a correct diagnosis. Content Type Journal ArticleCategory Gynecolo...
Source: Archives of Gynecology and Obstetrics - October 24, 2009 Category: OBGYN Tags: Archives of Gynecology and Obstetrics Source Type: journals

Vitamin K deficiency bleeding in cholestatic infants with alpha-1-antitrypsin deficiencyEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
Conclusions: The risk of VKD bleeding in breastfed infants with A1AD was high and did not correlate with serum level of conjugated bilirubin at diagnosis. A similar absolute risk was previously reported in breastfed infants with biliary atresia under the same prophylactic regimen. This confirms that—without adequate prophylaxis—the risk of VKD bleeding is uniformly high in exclusively breastfed infants with cholestatic jaundice, irrespective of underlying aetiology. (Source: Archives of Disease in Childhood - Fetal and Neonatal Edition)
Source: Archives of Disease in Childhood - Fetal and Neonatal Edition - October 21, 2009 Category: Perinatology & Neonatology Authors: van Hasselt, P M, Kok, K, Vorselaars, A D M, van Vlerken, L, Nieuwenhuys, E, de Koning, T J, de Vries, R A, Houwen, R H J Tags: Pancreas and biliary tract, Childhood nutrition, Malnutrition, Reproductive medicine, Infant nutrition (including breastfeeding), Rheumatology Original articles Source Type: journals

Neutrophil Elastase Increases Airway Epithelial Nonheme Iron LevelsEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
Alpha-1-antitrypsin (A1AT) deficiency is characterized by increased neutrophil elastase (NE) activity and oxidative stress in the lung. We hypothesized that NE exposure generates reactive oxygen species by increasing lung nonheme iron. To test this hypothesis, we measured bronchoalveolar lavage (BAL) iron and ferritin levels, using inductively coupled plasma (ICP) optical emission spectroscopy and an ELISA, respectively, in A1AT-deficient patients and healthy subjects. To confirm the role of NE in regulating lung iron homeostasis, we administered intratracheally NE or control buffer to rats and measured BAL and lung iron a...
Source: Clinical and Translational Science - October 5, 2009 Category: Biomedical Science Authors: Bernard M. Fischer, Denise A. Lopez Domowicz, Shuo Zheng, Jamie L. Carter, N. Gerry McElvaney, Clifford Taggart, James R. Lehmann, Judith A. Voynow, Andrew J. Ghio Source Type: journals

Development of Predictive Models for Airflow Obstruction in Alpha-1-Antitrypsin DeficiencyEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
Alpha-1-antitrypsin deficiency is a genetic condition associated with severe, early-onset chronic obstructive pulmonary disease (COPD). However, there is significant variability in lung function impairment among persons with the protease inhibitor ZZ genotype. Early identification of persons at highest risk of developing lung disease could be beneficial in guiding monitoring and treatment decisions. Using a multicenter, family-based study sample (2002–2005) of 372 persons with the protease inhibitor ZZ genotype, the authors developed prediction models for forced expiratory volume in 1 second (FEV1) and the presence o...
Source: American Journal of Epidemiology - October 1, 2009 Category: Epidemiology Authors: Castaldi, P. J., DeMeo, D. L., Kent, D. M., Campbell, E. J., Barker, A. F., Brantly, M. L., Eden, E., McElvaney, N. G., Rennard, S. I., Stocks, J. M., Stoller, J. K., Strange, C., Turino, G., Sandhaus, R. A., Griffith, J. L., Silverman, E. K. Tags: RESEARCH-ARTICLE Source Type: journals

{alpha}1-Antitrypsin deficiency: best clinical practice [Best practice]Email this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
1-Antitrypsin (AAT), a 52 kDa plasma protein, is produced mainly in the liver. It is the most abundant circulating serine proteinase inhibitor (serpin). It has also previously been called protease inhibitor to reflect its function as a general inhibitor of serine proteases. Its main physiological role is to inhibit neutrophil elastase and it contributes to the innate immune system as an anti-inflammatory protein. Severe AAT deficiency is most prevalent in northern Europeans affecting about 1 in 3000 of the population. AAT deficiency predisposes individuals who smoke to developing pulmonary emphysema in the fourth–fif...
Source: Journal of Clinical Pathology - September 25, 2009 Category: Pathology Authors: Kalsheker, N A Tags: Liver disease, Immunology (including allergy), Biological agents Best practice Source Type: journals

[Best practice] {alpha}1-Antitrypsin deficiency: best clinical practiceEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
1-Antitrypsin (AAT), a 52 kDa plasma protein, is produced mainly in the liver. It is the most abundant circulating serine proteinase inhibitor (serpin). It has also previously been called protease inhibitor to reflect its function as a general inhibitor of serine proteases. Its main physiological role is to inhibit neutrophil elastase and it contributes to the innate immune system as an anti-inflammatory protein. Severe AAT deficiency is most prevalent in northern Europeans affecting about 1 in 3000 of the population. AAT deficiency predisposes individuals who smoke to developing pulmonary emphysema in the fourth–fif...
Source: Journal of Clinical Pathology - September 24, 2009 Category: Pathology Authors: Kalsheker, N A Tags: Liver disease, Immunology (including allergy), Biological agents Best practice Source Type: journals

Sustained transgene expression despite T lymphocyte responses in a clinical trial of rAAV1-AAT gene therapy [Medical_Sciences]Email this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
Alpha-1 antitrypsin (AAT) deficiency is well-suited as a target for human gene transfer. We performed a phase 1, open-label, dose-escalation... (Source: Proceedings of the National Academy of Sciences)
Source: Proceedings of the National Academy of Sciences - September 21, 2009 Category: Science Authors: Brantly, M. L., Chulay, J. D., Wang, L., Mueller, C., Humphries, M., Spencer, L. T., Rouhani, F., Conlon, T. J., Calcedo, R., Betts, M. R., Spencer, C., Byrne, B. J., Wilson, J. M., Flotte, T. R. Tags: Medical_Sciences Source Type: journals

Is lysyl oxidase-like protein-1, alpha-1 antitrypsin, and neutrophil elastase site specific in pelvic organ prolapse?Email this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
Conclusions  ATT, NE, and LOXl-1 expression was variable among different biopsy sites in the vagina. No consistent pattern was present when the subjects were grouped by the most prominent defect. We recommend careful consideration of biopsy sites in future studies on POP to enhance reproducibility of data. Content Type Journal ArticleCategory Original ArticleDOI 10.1007/s00192-009-0905-yAuthors Weng Chi Man, Stanford University School of Medicine Department of Obstetrics and Gynecology 300 Pasteur Drive, HH-333 Stanford CA 94305-5317 USAJason Yen-Ping Ho, Stanford University School of Medicine Departme...
Source: International Urogynecology Journal - September 18, 2009 Category: OBGYN Tags: International Urogynecology Journal Source Type: journals

Kamada Presents Data On Inhaled Alpha-1 Antitrypsin In Cystic Fibrosis Patients At The European Respiratory Society Meeting 2009, ViennaEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
Kamada, a bio-pharmaceutical company engaged in the development, manufacturing and marketing of specialty life-saving therapeutics, announced that data on its next-generation alpha-1 antitrypsin (Inhaled-AAT) in cystic fibrosis patients was presented, at the Annual Congress of the European Respiratory Society (ERS) 2009, Vienna. (Source: Health News from Medical News Today)
Source: Health News from Medical News Today - September 16, 2009 Category: Consumer Health News Tags: Cystic Fibrosis Source Type: news

Kamada Presents Data on Inhaled Alpha-1 Antitrypsin in Cystic Fibrosis Patients at the European Respiratory Society Meeting 2009, ViennaEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
NESS ZIONA, Israel--(BUSINESS WIRE)--Sep 14, 2009 - Kamada, a bio-pharmaceutical company engaged in the development, manufacturing and marketing of specialty life-saving therapeutics, announced that data on its next-generation alpha-1... (Source: Drugs.com - Clinical Trials)
Source: Drugs.com - Clinical Trials - September 15, 2009 Category: Pharmaceuticals Source Type: clinical trials

High burden of acute exacerbation found in COPD with AATDEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
The vast majority of patients with chronic obstructive pulmonary disease and concurrent alpha-1 antitrypsin deficiency will experience at least one exacerbation per year, despite augmentation therapy, study findings show. (Source: MedWire News - Respiratory)
Source: MedWire News - Respiratory - September 10, 2009 Category: Respiratory Medicine Source Type: news

Z and Mmalton-1-antitrypsin deficiency-associated hepatocellular carcinoma: a genetic studyEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
The histological hallmark of [alpha]-1-antitrypsin deficiency (AATD) is the presence of periodic acid-Schiff diastase (PASD)-resistant positive globules in hepatocytes, with a heterogeneous distribution. It is noteworthy that hepatocellular carcinoma (HCC) arises specifically from the AAT-negative areas but the reason for this remains unclear. To determine whether the different distribution of AAT globules within neoplastic and non-neoplastic hepatocytes is the result of a self-induced correction of the genetic defect. Two HCV-positive patients with AATD-associated HCC were studied. One patient harboured a compound heteroz...
Source: Liver International - September 9, 2009 Category: Gastroenterology Authors: Paola Francalanci, Filippo M. Santorelli, Simona Saccani, Maria F. Bonetti, Daniela Medicina, Pierpaolo Coni, Gavino Faa, Francesco Callea Source Type: journals

Gene variant heightens risk of severe liver diseaseEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
Scientists at the University of North Carolina at Chapel Hill have discovered a genetic risk factor for severe liver disease in people with cystic fibrosis. Those who carry a particular variant of the SERPINA1 gene (also known as alpha-1-antitrypsin or alpha-1-antiprotease) are five times more likely to develop cirrhosis and other liver complications than patients who carry the normal version of the gene........ (Source: Medicineworld.org: New Article Alert)
Source: Medicineworld.org: New Article Alert - September 9, 2009 Category: Consumer Health News Source Type: info

Development of Predictive Models for Airflow Obstruction in Alpha-1-Antitrypsin Deficiency.Email this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
Alpha-1-antitrypsin deficiency is a genetic condition associated with severe, early-onset chronic obstructive pulmonary disease (COPD). However, there is significant variability in lung function impairment among persons with the protease inhibitor ZZ genotype. Early identification of persons at highest risk of developing lung disease could be beneficial in guiding monitoring and treatment decisions. Using a multicenter, family-based study sample (2002-2005) of 372 persons with the protease inhibitor ZZ genotype, the authors developed prediction models for forced expiratory volume in 1 second (FEV(1)) and the presence o...
Source: Am J Epidemiol - September 1, 2009 Category: Epidemiology Authors: Castaldi PJ, Demeo DL, Kent DM, Campbell EJ, Barker AF, Brantly ML, Eden E, McElvaney NG, Rennard SI, Stocks JM, Stoller JK, Strange C, Turino G, Sandhaus RA, Griffith JL, Silverman EK Tags: Am J Epidemiol Source Type: journals

Exploring the optimum approach to the use of CT densitometry in a randomised placebo-controlled study of augmentation therapy in alpha 1-antitrypsin deficiencyEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
Conclusions: PD15 is the most sensitive index of emphysema progression and of treatment modification. Targeted sampling may be more sensitive than whole lung analysis.Trial registration: Registered in ClinicalTrials.gov as 'Antitrypsin (AAT) to Treat Emphysema in AAT-Deficient Patients'; ClinicalTrials.gov Identifier: NCT00263887. (Source: Respiratory Research)
Source: Respiratory Research - August 12, 2009 Category: Respiratory Medicine Authors: David ParrAsger DirksenEeva PiitulainenChunqin DengMarion WenckerRobert Stockley Source Type: journals

Gene therapy hope for people with alpha-1 antitrypsin deficiencyEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
Patients with a genetic deficiency that increases the risk for emphysema could soon benefit from therapy to “replace” the defective gene, say researchers. (Source: MedWire News - Respiratory)
Source: MedWire News - Respiratory - August 12, 2009 Category: Respiratory Medicine Source Type: news

Gene Therapy Trial Succeeds In Boosting Protective Protein In Patients With Hereditary Lung DiseaseEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
Gene therapy researchers have safely given new, functional genes to patients with a hereditary defect that can lead to fatal lung and liver diseases, according to clinical trial findings. Three patients, apparently for the first time in their lives, produced trace amounts of the protective form of a protein called alpha-1 antitrypsin for up to one year, a potential step toward a gene therapy for about 100,000 Americans with alpha-1 antitrypsin deficiency. (Source: ScienceDaily Headlines)
Source: ScienceDaily Headlines - August 11, 2009 Category: Science Source Type: news

New genes at work in patients with hereditary lung diseaseEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
(University of Massachusetts Medical School) Researchers at the University of Massachusetts Medical School and the University of Florida in Gainesville have safely given new, functional genes to patients with alpha-1 antitrypsin deficiency, a hereditary defect that can lead to fatal lung and liver diseases. (Source: EurekAlert! - Medicine and Health)
Source: EurekAlert! - Medicine and Health - August 10, 2009 Category: Global & Universal Source Type: news

New genes at work in patients with hereditary lung diseaseEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
(University of Florida) Gene therapy researchers have safely given new, functional genes to patients with a hereditary defect that can lead to fatal lung and liver diseases, according to clinical trial findings. Three patients, apparently for the first time in their lives, produced trace amounts of the protective form of a protein called alpha-1 antitrypsin for up to one year, a potential step toward a gene therapy for about 100,000 Americans with alpha-1 antitrypsin deficiency. (Source: EurekAlert! - Medicine and Health)
Source: EurekAlert! - Medicine and Health - August 10, 2009 Category: Global & Universal Source Type: news

Angiostatic factors normally restrict islet endothelial cell proliferation and migration: implications for islet transplantationEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
In conclusion, VEGF may attract recipient blood vessels towards intrahepatically transplanted islets, but intra-islet vascular expansion is hampered by angiostatic factors present within the islets and the islet endothelium. Inhibition of angiostatic factors early after transplantation may provide a strategy to restore the islet vascular network and improve islet graft function. (Source: Transplant International)
Source: Transplant International - August 6, 2009 Category: Transplant Surgery Authors: Åsa Johansson, Johan Olerud, Magnus Johansson, Per-Ola Carlsson Source Type: journals

Alpha-1 Foundation Research OpportunitiesEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
The objective of this grant is to provide support for excellent Alpha-1 Antitrypsin (AAT) Deficiency-related research projects that have been submitted to and approved by the National Institutes of Health (NIH), but cannot be supported by the available NIH funds. Thus, this award complements the granting mechanism of the NIH for projects of potential interest to individuals served by the Alpha-1 Foundation. Funds provided by the Alpha-1 Foundation through this program are intended to lead to the development of sufficient preliminary data to make AAT-related applications highly competitive in the NIH review process. Informa...
Source: ScanGrants feed - August 4, 2009 Category: Research Authors: Alpha-1 Foundation Source Type: funding

Kamada's Biologics License Application for IV-AAT to Treat Alpha-1 Antitrypsin Deficiency is Accepted for Review by the US Food and Drug AdministrationEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
NESS ZIONA, Israel--(BUSINESS WIRE)--Aug 4, 2009 - Kamada, a bio-pharmaceutical company engaged in the development, manufacturing and marketing of specialty life-saving therapeutics, announced today that the United States Food and... (Source: Drugs.com - New Drug Applications)
Source: Drugs.com - New Drug Applications - August 4, 2009 Category: Drugs & Pharmacology Source Type: news

Chlorhexidine prevents hypochlorous acid-induced inactivation of alpha-1-antitrypsin (alpha-1-AT).Email this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
1. Chlorhexidine digluconate has been used as a topical antiseptic in the treatment of acne vulgaris and periodontitis. The acute phase of these diseases involves neutrophilic infiltration. Neutrophil activation and recruitment to inflammatory sites are crucial in both protection against bacterial infections and induction of hystotoxic damage. Activated neutrophils release several enzymes (elastase and myeloperoxidase [MPO]), which contribute to tissue injury through the direct toxic activity, the generation of oxidants and the inactivation of protective factors, such as alpha-1-antitrypsin (alpha-1-AT). In the present...
Source: Clinical and Experimental Pharmacology and Physiology - August 3, 2009 Category: Drugs & Pharmacology Authors: Montecucco F, Bertolotto M, Ottonello L, Pende A, Dapino P, Quercioli A, Mach F, Dallegri F Tags: Clin Exp Pharmacol Physiol Source Type: journals

Impact of rituximab-associated B-cell defects on West Nile virus meningoencephalitis in solid organ transplant recipientsEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
We describe a case of a patient with alpha-1-antitrypsin deficiency who underwent single lung transplantation in 2005 and was maintained on tacrolimus, cytoxan and prednisone. More recently, she had received two courses of rituximab for recurrent A2[ndash]A3 grade rejection with concomitant capillaritis and presented six months later with rapid, fulminant WNV meningoencephalitis. Her diagnosis was made by cerebrospinal fluid (CSF) PCR but serum and CSF WNV IgM and IgG remained negative. She received WNV-specific hyperimmune globulin (Omr-Ig-Am®) through a compassionate protocol. She experienced a rapidly progressive and d...
Source: Clinical Transplantation - July 30, 2009 Category: Transplant Surgery Authors: Marilyn E. Levi, Dianna Quan, Joseph T. Ho, B. K. Kleinschmidt-DeMasters, Kenneth L. Tyler, Todd J. Grazia Source Type: journals

Oxidized {alpha}1-antitrypsin stimulates the release of monocyte chemotactic protein-1 from lung epithelial cells: potential role in emphysemaEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
1-Antitrypsin (AT) is a major elastase inhibitor within the lung. Oxidation of critical methionine residues in AT generates oxidized AT (Ox-AT), which has a greatly diminished ability to inhibit neutrophil elastase. This process may contribute to the pathogenesis of chronic obstructive pulmonary disease (COPD) by creating a functional deficiency of AT permitting lung destruction. We show here that Ox-AT promotes release of human monocyte chemoattractant protein-1 (MCP-1) and IL-8 from human lung type epithelial cells (A549) and normal human bronchial epithelial (NHBE) cells. Native, cleaved, polymeric AT and secretory leuk...
Source: AJP: Lung Cellular and Molecular Physiology - July 22, 2009 Category: Respiratory Medicine Authors: Li, Z., Alam, S., Wang, J., Sandstrom, C. S., Janciauskiene, S., Mahadeva, R. Tags: ARTICLES Source Type: journals

Activity-based mass spectrometric characterization of proteases and inhibitors in human salivaEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
Proteases present in oral fluid effectively modulate the structure and function of some salivary proteins and have been implicated in tissue destruction in oral disease. To identify the proteases operating in the oral environment, proteins in pooled whole saliva supernatant were separated by anion-exchange chromatography and individual fractions were analyzed for proteolytic activity by zymography using salivary histatins as the enzyme substrates. Protein bands displaying proteolytic activity were particularly prominent in the 50-75 kDa region. Individual bands were excised, in-gel trypsinized and subjected to LC/ESI-MS/MS...
Source: Proteomics. Clinical Applications - July 8, 2009 Category: Biochemistry Authors: Xiuli Sun, Erdjan Salih, Frank G. Oppenheim, Eva J. Helmerhorst Tags: Research Articles Source Type: journals

Proteomic analysis of circulating immune complexes in juvenile idiopathic arthritis reveals disease-associated proteinsEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
In this study, the circulating immune complex proteome was examined to elucidate disease-associated proteins that are overexpressed in patients with an aggressive, and at times destructive, disease phenotype. To solve this proteome, circulating immune complexes were isolated from the sera of patients with chronic, erosive or early-onset, aggressive disease and from patients in medical remission or healthy controls subsequent to protein separation by 2-DE. Thirty-seven protein spots were overexpressed in the circulating immune complexes of the aggressive disease groups as compared to controls, 28 of which have been confiden...
Source: Proteomics. Clinical Applications - July 8, 2009 Category: Biochemistry Authors: Jason M. Low, Anil K. Chauhan, David S. Gibson, Mengmeng Zhu, Sixue Chen, Madeleine E. Rooney, Michael J. Ombrello, Terry L. Moore Tags: Research Articles Source Type: journals

Serum trypsin inhibitory capacity in hemodialysis patients.Email this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
It has been established that overproduction of reactive oxygen species (ROS) occurs during hemodialysis causing oxidation of proteins. Alpha-1-antitrypsin is the major circulating anti-protease which contains methionine in the active site. The aim of the present study was to measure the level of serum trypsin inhibitory capacity (sTIC) in hemodialysis patients. This case-control study was performed in 52 hemodialysis patients and 49 healthy controls. sTIC was measured by enzymatic assay. The sTIC was significantly (P< 0.001) lower in hemodialysis patients (1.87 +/- 0.67 micromol/min/mL) than healthy controls (2.83 +...
Source: Saudi Journal of Kidney Diseases and Transplantation - June 30, 2009 Category: Urology & Nephrology Authors: Hashemi M, Mehrabifar H, Homayooni F, Naderi M, Montazerifar F, Ghavami S Tags: Saudi J Kidney Dis Transpl Source Type: journals

[Biochemical and molecular diagnosis of alpha 1 antitrypsin deficiency in a Tunisian family.]Email this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
Our study investigated alpha 1 antitrypsin deficiency (AATD) diagnosis in a family originated from central Tunisia and showing a familial history of asthma. Biochemical and genetic diagnosis for AATD was performed according to current diagnostic standards. AAT level quantification in affected individuals showed plasma AAT levels consistent with intermediate AATD (ranged from 0.91 to 1.04 g/L). The molecular analysis was assessed using the genotyping of the most prevalent PI*S and PI*Z SERPINA1 mutations and the sequencing of AAT coding exons for rare AATD variants detection. No PI*S or PI*Z deficient variants were seen...
Source: Annales de Biologie Clinique - June 30, 2009 Category: Biochemistry Authors: Denden S, Braham W, Amri F, Lakhdar R, Lefranc G, Knani J, Chibani JB, Khelil AH Tags: Ann Biol Clin (Paris) Source Type: journals

High intestinal IgA associates with reduced risk of IgE-associated allergic diseasesEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
Kukkonen K, Kuitunen M, Haahtela T, Korpela R, Poussa T, Savilahti E. High intestinal IgA associates with reduced risk of IgE-associated allergic diseases.Pediatr Allergy Immunol 2009.© 2009 John Wiley & Sons A/S Development of oral tolerance and its stimulation by probiotics are still incomprehensible. Microbial stimulation of the gut may induce a subtle inflammation and induce secretion of mucosal IgA, which participates in antigen elimination. In a cohort of allergy-prone infants receiving probiotics and prebiotics or placebo we studied intestinal IgA and inflammation in the development of eczema, food allergy, asthma,...
Source: Pediatric Allergy and Immunology - June 28, 2009 Category: Allergy & Immunology Authors: Kaarina Kukkonen, Mikael Kuitunen, Tari Haahtela, Riitta Korpela, Tuija Poussa, Erkki Savilahti Source Type: journals

Protein profiling of low-density lipoprotein from obese subjectsEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
Although obesity and high levels of low-density lipoprotein (LDL) are well-known risk factors for cardiovascular disease, the precise role(s) of different LDL constituents in obesity has not been explored. In the present study, we compared the LDL proteome of healthy control adults (body mass index30). LDL was isolated by density-gradient ultracentrifugation and proteins were separated with 2-D PAGE, quantified, and identified by peptide mass fingerprinting using MALDI-TOF MS. A new LDL-associated protein was identified as transthyretin and found to be significantly more abundant in LDL from the obese subjects. In addition...
Source: Proteomics. Clinical Applications - June 23, 2009 Category: Biochemistry Authors: Helen Karlsson, Harriet Mörtstedt, Helen Lindqvist, Christer Tagesson, Mats Lindahl Tags: Research Articles Source Type: journals

[Mechanisms Of Signal Transduction] Selenoprotein S/SEPS1 Modifies Endoplasmic Reticulum Stress in Z Variant {alpha}1-Antitrypsin DeficiencyEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
Z 1-antitrypsin (ZAAT) deficiency is a disease associated with emphysematous lung disease and also with liver disease. The liver disease of AAT deficiency is associated with endoplasmic reticulum (ER) stress. SEPS1 is a selenoprotein that, through a chaperone activity, decreases ER stress. To determine the effect of SEPS1 on ER stress in ZAAT deficiency, we measured activity of the grp78 promoter and levels of active ATF6 as markers of the unfolded protein response in HepG2 cells transfected with the mutant form of AAT, a ZAAT transgene. We evaluated levels of NFB activity as a marker of the ER overload response. To determ...
Source: Journal of Biological Chemistry - June 11, 2009 Category: Chemistry Authors: Kelly, E., Greene, C. M., Carroll, T. P., McElvaney, N. G., O'Neill, S. J. Tags: Mechanisms Of Signal Transduction Source Type: journals

Oxidized alpha-1 antitrypsin stimulates the release of monocyte chemotactic protein-1 from lung epithelial cells: potential role in emphysema.Email this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
Alpha-1 antitrypsin (AT) is a major elastase inhibitor within the lung. Oxidation of critical methionine residues in AT generates Oxidized AT (Ox-AT) which has a greatly diminished ability to inhibit neutrophil elastase. This process may contribute to the pathogenesis of chronic obstructive pulmonary disease (COPD) by creating a functional deficiency of AT permitting lung destruction. We show here that Ox-AT promotes release of human monocyte chemoattractant protein-1 (MCP-1) and interleukin-8 (IL-8) from human lung type epithelial cells (A549) and normal human bronchial epithelial (NHBE) cells. Unoxidized and oxidized...
Source: American Journal of Physiology. Lung Cellular and Molecular Physiology - June 11, 2009 Category: Cytology Authors: Li Z, Alam S, Wang J, Sandstrom CS, Janciauskiene S, Mahadeva R Tags: Am J Physiol Lung Cell Mol Physiol Source Type: journals

Kamada Reports Positive Proof-of-Concept Data for Inhaled Alpha-1 Antitrypsin in BronchiectasisEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
NESS ZIONA, Israel--(BUSINESS WIRE)--Jun 9, 2009 - Kamada (TASE:KMDA), a bio-pharmaceutical company engaged in the development, manufacturing and marketing of specialty life-saving therapeutics, announced today positive proof of concept data from a... (Source: Drugs.com - Clinical Trials)
Source: Drugs.com - Clinical Trials - June 9, 2009 Category: Pharmaceuticals Source Type: clinical trials

Expression of liver-specific markers in naïve adipose-derived mesenchymal stem cellsEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
Increasing evidence suggests that adipose tissue contains mesenchymal stem cells (MSC) that possess the ability to transdifferentiate into other cell types including hepatocytes, similar to bone marrow-derived stem cells. The existence of precommitted cells in the MSC population may explain transdifferentiation. Our aim was to identify a population of putative hepatocyte-like precursor cells in human adipose tissue. We analysed the 'basal' hepatic potential of undifferentiated, naïve human adipose-derived mesenchymal stem cells (hADMSC). hADMSC were isolated from human adipose tissue and characterized for cell surface mar...
Source: Liver International - June 9, 2009 Category: Gastroenterology Authors: Romy Zemel, Larisa Bachmetov, Dean Ad-El, Amir Abraham, Ran Tur-Kaspa Source Type: journals

Cluster headache and alpha 1-antitrypsin deficiencyEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
Summ O, Gregor N, Marziniak M, Gralow I, Husstedt IW & Evers S. Cluster headache and alpha 1-antitrypsin deficiency. Cephalalgia 2009. London. ISSN 0333-1024 Little is known about the pathophysiology of cluster headache (CH), one of the most debilitating primary headaches. Interestingly, associations of lung affecting diseases or lifestyle habits such as smoking and sleep apnoea syndrome and CH have been described. Certain genotypes for alpha 1-antitrypsin ([alpha]1-AT) are considered risk factors for emphysema. Our aim was to investigate possible associations between common genotypes of the SERPINA1 gene and CH. Our study...
Source: Cephalalgia - June 8, 2009 Category: Neurology Authors: O Summ, N Gregor, M Marziniak, I Gralow, IW Husstedt, S Evers Source Type: journals

Interactions of alpha(1)-proteinase inhibitor with small ligands of therapeutic potential: binding with retinoic acid.Email this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
The objective of this study was to evaluate the potential of alpha(1)-PI to bind small non-peptide ligands of pharmaceutical interest that may attain additional properties to currently available alpha(1)-PI therapeutic preparations. Among putative ligands of bio-medical interest examined in this study, all-trans retinoic acid (RA) was selected due to its recently proposed roles in the lungs, and as an efficient optical probe. The results of this study, including absorption spectroscopy data, fluorescence quenching and the protein-induced chirality of the visible circular dichroism strongly suggest that alpha(1)-PI does bin...
Source: Amino Acids - June 2, 2009 Category: Biochemistry Authors: Karnaukhova E Tags: Amino Acids Source Type: journals

Kamada Submits U.S. Biologics License Application for the First Liquid, Ready to Use AAT for the Treatment of Alpha-1 Antitrypsin DeficiencyEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
NESS ZIONA, Israel--(BUSINESS WIRE)--Jun 2, 2009 - Kamada (TASE:KMDA) – a bio-pharmaceutical company engaged in the development, manufacturing and marketing of specialty life-saving therapeutics, announced that it has submitted a Biologics License... (Source: Drugs.com - New Drug Applications)
Source: Drugs.com - New Drug Applications - June 2, 2009 Category: Drugs & Pharmacology Source Type: news

Exacerbations in subjects with alpha-1 antitrypsin deficiency receiving augmentation therapyEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
Conclusion: COPD exacerbations occur frequently and are associated with significant disease burden in subjects with AATD receiving augmentation therapy. (Source: Respiratory Medicine)
Source: Respiratory Medicine - May 24, 2009 Category: Respiratory Medicine Authors: Michael A. Campos, Saleh Alazemi, Guoyan Zhang, Adam Wanner, Matthias Salathe, Horst Baier, Robert A. Sandhaus Tags: Original Articles Source Type: journals

Kamada Presents New Data on its Alpha-1 Antitrypsin at the American Thoracic Society Annual MeetingEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
<p><dateline></dateline>NESS ZIONA, Israel--(BUSINESS WIRE)--May 19, 2009 - Kamada, a bio-pharmaceutical company engaged in the development, manufacturing and marketing of specialty life-saving therapeutics, today announced that... (Source: Drugs.com - Clinical Trials)
Source: Drugs.com - Clinical Trials - May 19, 2009 Category: Pharmaceuticals Source Type: clinical trials

Alpha-1-antitrypsin deficiency in Madeira (Portugal): The highest prevalence in the worldEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
Summary: Alpha-1-antitrypsin (AAT) deficiency is a common genetic disease which affects both lung and liver. Early diagnosis can help asymptomatic patients to adjust their lifestyle choices in order to reduce the risk of Chronic Obstructive Pulmonary Disease (COPD). The determination of this genetic deficiency prevalence in Madeira Island (Portugal) population is important to clarify susceptibility and define the relevance of performing genetic tests for AAT on individuals at risk for COPD.Two hundred samples of unrelated individuals from Madeira Island were genotyped for the two most common AAT deficiency alleles, PI*S an...
Source: Respiratory Medicine - May 17, 2009 Category: Respiratory Medicine Authors: Carla Spínola, Jácome Bruges-Armas, Conceição Pereira, António Brehm, Hélder Spínola Tags: Original Articles Source Type: journals

Morbid Obesity and Inflammation: A Prospective Study After Adjustable Gastric Banding SurgeryEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
Conclusions  Our results indicate that amelioration of inflammatory markers after LAGB does not seem to negatively impact nutritional status following weight reduction surgery. However, careful attention should be driven to serum IgA. Adequacy of nutritional intake and complete serial laboratory measurements should be always included in the required life-long follow-up of patients surgically treated for morbid obesity. Content Type Journal ArticleCategory Clinical ResearchDOI 10.1007/s11695-009-9848-0Authors Renata Ramalho, University of Porto Immunology, Faculty of Medicine Alameda Prof. Hernâni Mon...
Source: Obesity Surgery - May 14, 2009 Category: Surgery Tags: Obesity Surgery Source Type: journals

Mortality in alpha-1-antitrypsin deficiency in the United KingdomEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
Summary: Background: Four hundred and eighty-eight PiZ alpha-1-antitrypsin deficient patients, who had joined the UK registry over a 9-year period, were followed in an observational study to determine mortality. None had received A1AT augmentation therapy.Methods: Cause of death was confirmed from death certification and medical records. Patients were censored according to length of time on the program or until they withdrew from the program.Results: There were 56 deaths of which 30 were attributed to respiratory causes. Of the remaining 26 deaths, 4 were due to complications from lung transplant, 6 due to liver disease (i...
Source: Respiratory Medicine - May 13, 2009 Category: Respiratory Medicine Authors: Paul Dawkins, Alice Wood, Peter Nightingale, Robert Stockley Tags: Original Articles Source Type: journals

Lung Volume Reduction Surgery for Patients with Alpha-1 Antitrypsin Deficiency EmphysemaEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
Alpha-1 antitrypsin deficiency (A1AD) is a rare genetic disorder characterized by early-onset emphysema and, rarely, liver disease and vasculitis. In the lungs, unopposed and enhanced elastase activity results in accelerated parenchymal destruction leading to emphysematous changes predominantly in the lower lung fields. Medical treatment includes standard therapies for emphysema and so-called “augmentation therapy” using purified pooled plasma alpha-1 antitrypsin. Surgical options include lung transplantation and lung volume reduction surgery. The option of lung volume reduction surgery potentially provides palliation ...
Source: Thoracic Surgery Clinics - April 30, 2009 Category: Cardiovascular & Thoracic Surgery Authors: James M. Donahue, Stephen D. Cassivi Source Type: journals

Public Workshop: Improving Endpoints, Improving CareEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
Alpha-1 Antitrypsin Augmentation Therapy and Clinical Trials (Source: What's New at CBER)
Source: What's New at CBER - April 28, 2009 Category: Biomedical Science Source Type: organizations