Reactive centre loop mutants of α-1-antitrypsin reveal position-specific effects on intermediate formation along the polymerization pathway

Reactive centre loop mutants of α-1-antitrypsin reveal position-specific effects on intermediate formation along the polymerization pathway

The common severe Z mutation (E342K) of α1-antitrypsin forms intracellular polymers that are associated with liver cirrhosis. The native fold of this protein is well-established and models have been proposed from crystallographic and biophysical data for the stable inter-molecular configu...

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Bibliographic Details
Main Authors: Imran Haq, James A. Irving, Sarah V. Faull, Jennifer A. Dickens, Adriana Ordóñez, Didier Belorgey, Bibek Gooptu, David A. Lomas
Format: Article
Language:English
Published: Portland Press, Biochemical Society 2013-06-01
Series:Bioscience Reports
Subjects:
cirrhosis
emphysema
FRET
intermediate
polymerization
serpin
Online Access:http://www.bioscirep.org/bsr/033/e046/bsr033e046.htm

Internet

http://www.bioscirep.org/bsr/033/e046/bsr033e046.htm

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