Alpha‐1 antitrypsin deficiency: a conformational disease associated with lung and liver manifestations

CM Greene, SDW Miller, T Carroll… - Journal of Inherited …, 2008 - Wiley Online Library
Summary Alpha‐1 antitrypsin (A1AT) is a serine anti‐protease produced chiefly by the liver.
A1AT deficiency is a genetic disorder characterized by serum levels of less than 11 μmol/L …

α1-antitrypsin deficiency: biological answers to clinical questions

RJ Coakley, C Taggart, S O'Neill… - The American journal of …, 2001 - Elsevier
ABSTRACT α1-antitrypsin (α1AT) deficiency is a common lethal hereditary disorder of white
persons of European descent. The condition is characterized by reduced serum levels of α …

Alpha-1-antitrypsin deficiency: current concepts

AT Mulgrew, CC Taggart, NG McElvaney - Lung, 2007 - Springer
Since the condition was first described four decades ago, alpha-1-antitrypsin (A1AT)
deficiency has served as a model for other disease processes. A1AT is the archetypal serpin …

Alpha-1-antitrypsin deficiency: Genetic variations, clinical manifestations and therapeutic interventions

YM Hazari, A Bashir, M Habib, S Bashir, H Habib… - … Research/Reviews in …, 2017 - Elsevier
Abstract Alpha-1-antitrypsin (AAT) is an acute phase secretory glycoprotein that inhibits
neutrophil proteases like elastase and is considered as the archetype of a family of …

[HTML][HTML] Alpha-1 antitrypsin deficiency

E Kelly, CM Greene, TP Carroll, NG McElvaney… - Respiratory Medicine …, 2011 - Elsevier
Objective To review the topic of alpha-1 antitrypsin (AAT) deficiency. Method Narrative
literature review. Results Much work has been carried out on this condition with many …

Molecular basis of the liver and lung disease associated with the α1-antitrypsin deficiency allele Mmalton

DT Curiel, MD Holmes, H Okayama, ML Brantly… - Journal of Biological …, 1989 - Elsevier
α1-Antitrypsin (α1AT) deficiency is characterized by reduced serum levels of α1AT and a risk
for the development of emphysema and liver disease. However, whereas there is an …

Molecular basis of alpha-1-antitrypsin deficiency

M Brantly, T Nukiwa, RG Crystal - The American journal of medicine, 1988 - Elsevier
Alpha-1-antitrypsin (A1AT) deficiency is an autosomal hereditary disorder associated with a
major reduction in serum A1AT levels. Clinically, A1AT deficiency is associated with …

Alpha-1-antitrypsin deficiency: biochemistry and clinical manifestations

DH Perlmutter - Annals of medicine, 1996 - Taylor & Francis
Alpha-1-antitrypsin (α1-AT) deficiency is a well known cause of emphysema in adults. A
subgroup of deficient individuals develops liver injury during infancy and childhood. In fact, it …

α1-Antitrypsin deficiency• 3: Clinical manifestations and natural history

M Needham, RA Stockley - Thorax, 2004 - thorax.bmj.com
α1-Antitrypsin deficiency • 3: Clinical manifestations and natural history | Thorax Skip to main
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[图书][B] Alpha 1-Antitrypsin Deficiency: Biology-Pathogenesis-Clinical Manifestations-Therapy

RG Crystal - 2014 - taylorfrancis.com
Providing a broad overview of basic and clinical aspects of alpha 1-antitrypsin (a 1AT)
deficiency, this up-to-date reference discusses the complex pathobiological processes …