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Discovery of an Inhibitor of Z-Alpha1 Antitrypsin Polymerization

Polymerization of the Z variant alpha-1-antitrypsin (Z-α1AT) results in the most common and severe form of α1AT deficiency (α1ATD), a debilitating genetic disorder whose clinical manifestations range from asymptomatic to fatal liver and/or lung disease. As the altered conformation of Z-α1AT and its...

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Detalles Bibliográficos
Autores principales: Berthelier, Valerie, Harris, Jason Brett, Estenson, Kasey Noel, Baudry, Jerome
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Public Library of Science 2015
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4427445/
https://www.ncbi.nlm.nih.gov/pubmed/25961288
http://dx.doi.org/10.1371/journal.pone.0126256