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SVIP regulates Z variant alpha-1 antitrypsin retro-translocation by inhibiting ubiquitin ligase gp78

Alpha-1 antitrypsin deficiency (AATD) is an inherited disorder characterized by early-onset emphysema and liver disease. The most common disease-causing mutation is a single amino acid substitution (Glu/Lys) at amino acid 342 of the mature protein, resulting in disruption of the 290–342 salt bridge...

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Detalles Bibliográficos
Autores principales: Khodayari, Nazli, Wang, Rejean liqun, Marek, George, Krotova, Karina, Kirst, Mariana, Liu, Chen, Rouhani, Farshid, Brantly, Mark
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Public Library of Science 2017
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5354272/
https://www.ncbi.nlm.nih.gov/pubmed/28301499
http://dx.doi.org/10.1371/journal.pone.0172983

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