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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 α(1)-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 th...

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Detalhes bibliográficos
Main Authors: Haq, Imran, Irving, James A., Faull, Sarah V., Dickens, Jennifer A., Ordóñez, Adriana, Belorgey, Didier, Gooptu, Bibek, Lomas, David A.
Formato: Artigo
Idioma:Inglês
Publicado em: Portland Press Ltd. 2013
Assuntos:
Acesso em linha:https://ncbi.nlm.nih.gov/pmc/articles/PMC3691886/
https://ncbi.nlm.nih.gov/pubmed/23659468
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1042/BSR20130038
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