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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...
| Autores principales: | , , , |
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| Formato: | Online Artículo Texto |
| Lenguaje: | English |
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Public Library of Science
2014
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| Materias: | |
| Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4151987/ https://www.ncbi.nlm.nih.gov/pubmed/25181470 http://dx.doi.org/10.1371/journal.pone.0102617 |