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...
Main Authors: | , , , , , , , |
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Format: | Article |
Language: | English |
Published: |
Portland Press, Biochemical Society
2013-06-01
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Series: | Bioscience Reports |
Subjects: | |
Online Access: | http://www.bioscirep.org/bsr/033/e046/bsr033e046.htm |