α‎<sub>1</sub>-Antitrypsin deficiency and the serpinopathies

David A. Lomas

in Oxford Textbook of Medicine

Published on behalf of Oxford University Press

Published in print May 2010 | ISBN: 9780199204854
Published online November 2012 | e-ISBN: 9780199570973 | DOI:

Series: Oxford Textbooks

α‎1-Antitrypsin deficiency and the serpinopathies

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α‎1-Antitrypsin is an acute phase glycoprotein synthesized by the liver that functions as an inhibitor of a range of proteolytic enzymes, most importantly neutrophil elastase. Severe plasma deficiency of α‎1-antitrypsin results from homozygocity for the Z allele, which causes the protein to undergo a conformational transition and form ordered polymers that are retained within hepatocytes as PAS-positive inclusions....

Chapter.  4505 words.  Illustrated.

Subjects: Clinical Medicine

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