Article
Alpha one antitrypsin deficiency: from gene to treatment.
Respiration; international review of thoracic diseases - 1 Jan 2007
Wood Alice M, Stockley Robert A
Abstract excerpt
Alpha1-antitrypsin deficiency is a genetic disorder which contributes to the development of chronic obstructive pulmonary disease, bronchiectasis, liver cirrhosis and panniculitis. The discovery of alpha1-antitrypsin and its function as an antiprotease led to the protease-antiprotease hypothesis, which goes some way to explaining the pathogenesis of emphysema. This article will review the clinical features of...
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