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Serpin polymerization and its role in disease—The molecular basis of α1-antitrypsin deficiency

✍ Scribed by Anja S. Knaupp; Stephen P. Bottomley

Publisher: John Wiley and Sons
Year: 2009
Tongue: English
Weight: 233 KB
Volume: 61
Category: Article
ISSN: 1521-6543
DOI: 10.1002/iub.127

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✦ Synopsis

Abstract

Protein aggregation is the cause of several human diseases. Understanding the molecular mechanisms involved in protein aggregation requires knowledge of the kinetics and structures populated during the reaction. Arguably, the best structurally characterized misfolding reaction is that of α~1~‐antitrypsin. α~1~‐Antitrypsin misfolding leads to both liver disease and emphysema and affect approximately 1 in 2000 of the population. This review will focus on the mechanism of α~1~‐antitrypsin misfolding and the development of potential therapeutic strategies. © 2008 IUBMB IUBMB Life, 61(1): 1–5, 2009

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