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The Z Mutation Alters the Global Structural Dynamics of α(1)-Antitrypsin

α(1)-Antitrypsin (α(1)AT) deficiency, the most common serpinopathy, results in both emphysema and liver disease. Over 90% of all clinical cases of α(1)AT deficiency are caused by the Z variant in which Glu342, located at the top of s5A, is replaced by a Lys which results in polymerization both in vi...

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Detalhes bibliográficos
Main Authors: Hughes, Victoria A., Meklemburg, Robert, Bottomley, Stephen P., Wintrode, Patrick L.
Formato: Artigo
Idioma:Inglês
Publicado em: Public Library of Science 2014
Assuntos:
Acesso em linha:https://ncbi.nlm.nih.gov/pmc/articles/PMC4151987/
https://ncbi.nlm.nih.gov/pubmed/25181470
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1371/journal.pone.0102617
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