[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 …

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 today: new insights in the immunological pathways

S Baraldo, E Balestro, E Bazzan, ME Tiné, D Biondini… - Respiration, 2016 - karger.com
More than 50 years ago, the observation that absence of the α 1 band from protein
electrophoresis is associated with severe emphysema established the link between α 1 …

Mysteries of α1-antitrypsin deficiency: emerging therapeutic strategies for a challenging disease

R Ghouse, A Chu, Y Wang… - Disease models & …, 2014 - journals.biologists.com
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 …

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: 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 …

Molecular pathology of α1-antitrypsin deficiency and its significance to clinical medicine

NA Kalsheker - QJM: An International Journal of Medicine, 1994 - academic.oup.com
Abstract α1-Antitrypsin (AAT) deficiency is associated with predisposition to developing liver
cirrhosis in early childhood, and chronic degenerative lung disease in early adult life. The …

Role of alpha‐1 antitrypsin in human health and disease

F De Serres, I Blanco - Journal of internal medicine, 2014 - Wiley Online Library
Abstract Alpha‐1 antitrypsin (AAT) deficiency is an under‐recognized hereditary disorder
associated with the premature onset of chronic obstructive pulmonary disease, liver cirrhosis …

Alpha-1 antitrypsin deficiency research and emerging treatment strategies: what's down the road?

FF Rahaghi - Therapeutic advances in chronic disease, 2021 - journals.sagepub.com
Intravenous infusion of alpha-1 antitrypsin (AAT) was approved by the United States Food
and Drug Administration (FDA) to treat emphysema associated with AAT deficiency (AATD) …

Alpha-1 antitrypsin deficiency: current perspective from genetics to diagnosis and therapeutic approaches

S Santangelo, S Scarlata, M L. Poeta… - Current medicinal …, 2017 - benthamdirect.com
Alpha-1 antitrypsin (A1AT) is a 52-kDa, acute phase glycoprotein encoded by the protease
inhibitor (PI) locus, located on the long arm of chromosome 14 (14q31-32.3). Its structure is …