Article
Reactive centre loop mutants of α-1-antitrypsin reveal position-specific effects on intermediate formation along the polymerization pathway.
Bioscience reports - 25 Jun 2013
Haq Imran, Irving James A, Faull Sarah V, Dickens Jennifer A, Ordóñez Adriana, Belorgey Didier, Gooptu Bibek, Lomas David A
Abstract excerpt
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 configuration that terminates the polymerization pathway. Despite these molecular 'snapshots', the details of the transition between...
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