نتایج جستجو برای: 1 antitrypsin a1at

تعداد نتایج: 2753541  

Journal: :Journal of pediatric gastroenterology and nutrition 2015
Jeffrey H Teckman Philip Rosenthal Robert Abel Lee M Bass Sonia Michail Karen F Murray David A Rudnick Daniel W Thomas Cathie Spino Ronen Arnon Paula M Hertel James Heubi Binita M Kamath Wikrom Karnsakul Kathleen M Loomes John C Magee Jean P Molleston Rene Romero Benjamin L Shneider Averell H Sherker Ronald J Sokol

OBJECTIVES α-1-Antitrypsin (A1AT) deficiency is a common genetic disease with an unpredictable and highly variable course. The Childhood Liver Disease Research and Education Network is a National Institutes of Health, multicenter, longitudinal consortium studying pediatric liver diseases, with the objective of prospectively defining natural history and identifying disease modifiers. METHODS L...

Journal: :Environmental Health Perspectives 1997
A Churg K Zay K Li

Mineral dust exposure can result in emphysema and chronic airflow obstruction. We postulated that dust-induced emphysema has a pathogenesis similar to that in cigarette smoke-induced emphysema, namely, excess release of proteolytic enzymes from dust-evoked inflammatory cells, and inactivation of alpha-1-antitrypsin (A1AT) by dust-catalyzed formation of oxidants. To test this theory we examined ...

Journal: :American journal of translational research 2009
Claudine L Bartels Angela L Marchetti W Edward Highsmith Gregory J Tsongalis

Alpha-1 antitrypsin (A1AT or AAT) is a serine protease inhibitor (PI) which, when present at low levels, can cause chronic obstructive pulmonary disease (COPD) and liver disease in both children and adults. Several mutations within the SERPINA1 gene have been found to cause this deficiency. The most common variants are PI*Z and PI*S, each caused by a single nucleotide polymorphism (SNP). We des...

2015
Jeffrey Teckman

A1AT deficiency has a complex pathophysiology, is highly variable in clinical course, and is under diagnosed. The association with chronic lung disease was first described by Eriksson and Laurell in 1963, and later, Sharp and colleagues recognized A1AT deficiency as a cause of liver disease. In 2013 an international meeting was held to review the intervening 50 years of basic and clinical scien...

2017
Fang Wang Simone Orioli Alan Ianeselli Giovanni Spagnolli Silvio a Beccara Anne Gershenson Pietro Faccioli Patrick L. Wintrode

Protein misfolding is implicated in many diseases, including the serpinopathies. For the canonical inhibitory serpin α1-antitrypsin (A1AT), mutations can result in protein deficiencies leading to lung disease and polymerization prone mutants can accumulate in hepatocytes leading to liver disease. Using all-atom simulations based on the recently developed Bias Functional algorithm we elucidate h...

Journal: :The Journal of general virology 2004
Tonino Alonzi Chiara Agrati Barbara Costabile Carla Cicchini Laura Amicone Claudio Cavallari Carlo Della Rocca Antonella Folgori Cristina Fipaldini Fabrizio Poccia Nicola La Monica Marco Tripodi

To assess the effects of constitutive hepatitis C virus (HCV) gene expression on liver, transgenic mice carrying the entire HCV open reading frame inserted in the alpha1 antitrypsin (A1AT) gene were generated. Expression of A1AT/HCV mRNA was found to be mainly limited to perivascular areas of the liver as indicated by in situ hybridization analysis. HCV core protein was detected in Western blot...

ژورنال: :iranian red crescent medical journal 0
bita geramizadeh department of pathology, transplant research center, shiraz university of medical sciences, shiraz, ir iran; transplant research center, shiraz university of medical sciences, shiraz, ir iran; department of pathology, transplant research center, shiraz university of medical sciences, shiraz, ir iran. tel: +98-7116474331, fax: +98-7116474331 zahra jowkar department of pathology, transplant research center, shiraz university of medical sciences, shiraz, ir iran leila karami department of pathology, transplant research center, shiraz university of medical sciences, shiraz, ir iran masoum masoumpour department of internal medicine, shiraz university of medical sciences, shiraz, ir iran samrad mehrabi department of internal medicine, shiraz university of medical sciences, shiraz, ir iran mohammad-ali ghayoumi department of internal medicine, shiraz university of medical sciences, shiraz, ir iran

background alpha-1 antitrypsin deficiency is a genetic disease which affects both lung and liver. this disease is a recognized factor for chronic obstructive pulmonary disease (copd). however its importance as the cause of copd in a country such as iran is unclear. objectives this study was conducted to find out the role of α-1 antitrypsin deficiency as a cause of copd in iranian patients. mate...

2013
Makito Miyake Shanti Ross Adrienne Lawton Myron Chang Yunfeng Dai Lourdes Mengual Antonio Alcaraz Evan Gomes Giacoia Steve Goodison Charles J Rosser

BACKGROUND In this study, we further investigated the association of two biomarkers, CCL18 and A1AT, with bladder cancer (BCa) and evaluated the influence of potentially confounding factors in an experimental model. METHODS In a cohort of 308 subjects (102 with BCa), urinary concentrations of CCL18 and A1AT were assessed by enzyme-linked immunosorbent assay (ELISA). In an experimental model, ...

2013
Qiaojun Wen Linda Y. Liu Ting Yang Cantas Alev Shuaibin Wu David K. Stevenson Guojun Sheng Atul J. Butte Xuefeng B. Ling

We sought to identify serological markers capable of diagnosing preeclampsia (PE). We performed serum peptide analysis (liquid chromatography mass spectrometry) of 62 unique samples from 31 PE patients and 31 healthy pregnant controls, with two-thirds used as a training set and the other third as a testing set. Differential serum peptide profiling identified 52 significant serum peptides, and a...

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