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

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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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Detalles Bibliográficos
Autores principales: Haq, Imran, Irving, James A., Faull, Sarah V., Dickens, Jennifer A., Ordóñez, Adriana, Belorgey, Didier, Gooptu, Bibek, Lomas, David A.
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Portland Press Ltd. 2013
Materias:
Original Paper
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3691886/
https://www.ncbi.nlm.nih.gov/pubmed/23659468
http://dx.doi.org/10.1042/BSR20130038
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