نتایج جستجو برای: α1 antitrypsin a1at

تعداد نتایج: 9172  

2014
Raafe Ghouse Andrew Chu Yan Wang David H. Perlmutter

The classical form of α1-antitrypsin deficiency (ATD) is an autosomal co-dominant disorder that affects ~1 in 3000 live births and is an important genetic cause of lung and liver disease. The protein affected, α1-antitrypsin (AT), is predominantly derived from the liver and has the function of inhibiting neutrophil elastase and several other destructive neutrophil proteinases. The genetic defec...

Journal: :American journal of physiology. Gastrointestinal and liver physiology 1999
Chaobin Hu David H Perlmutter

There is still relatively limited information about mechanisms of gene expression in enterocytes and mechanisms by which gene expression is regulated during enterocyte differentiation. Using the human intestinal epithelial cell line Caco-2, which spontaneously differentiates from a cryptlike to a villouslike enterocyte, we have previously shown that there is a marked increase in transcription o...

Journal: :Journal of the American Association of Nurse Practitioners 2021

ABSTRACT Cigarette smoking and poor air quality are the greatest risk factors for developing chronic obstructive pulmonary disease (COPD), but growing evidence indicates that genetic also affect predisposition to clinical expression of disease. With exception α1-antitrypsin deficiency (AATD), a rare autosomal recessive disorder is present in 1–3% individuals with COPD, no single gene associated...

Journal: :Anesthesiology 2021

Acute respiratory distress syndrome is characterized by hypoxemia, altered alveolar–capillary permeability, and neutrophil-dominated inflammatory pulmonary edema. Despite decades of research, an effective drug therapy for acute remains elusive. The ideal pharmacotherapy should demonstrate antiprotease activity target injurious pathways while maintaining host defense against infection. Furthermo...

2013
Zeyad J. El-Akawi Aymen M. Abu-awad Nabil A. Khouri

Background Alpha-1 antitrypsin (α1-AT) is a member of the serine protease inhibitors (serpins) family. Liver cells are the major source of synthesis and secretion of (α1-AT) into the blood. Moreover, it has been demonstrated that α1-AT is expressed and secreted by many types of malignant cells. Studies have indicated that serum levels of (α1-AT) increase in a good number of malignant diseases. ...

2014
BINGSHAN LIU GUOJUN LI XIAO WANG YANG LIU

This study aimed to explore the exact mechanism of the effect of a furin inhibitor on the migration and invasion of MG-63 and Saos-2 osteosarcoma cells. MG-63 and Saos-2 osteosarcoma cells were treated with regular culture medium in the presence or absence of 480 nM α1-antitrypsin Portland (α1-PDX). Wound-healing and Transwell assays were used for the detection of the effects of α1-PDX on MG-63...

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...

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