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Functional Rescue of Trafficking-Impaired ABCB4 Mutants by Chemical Chaperones

Multidrug resistance protein 3 (MDR3, ABCB4) is a hepatocellular membrane protein that mediates biliary secretion of phosphatidylcholine. Null mutations in ABCB4 gene give rise to severe early-onset cholestatic liver disease. We have previously shown that the disease-associated mutations p.G68R, p.G...

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Detalles Bibliográficos
Autores principales: Gordo-Gilart, Raquel, Andueza, Sara, Hierro, Loreto, Jara, Paloma, Alvarez, Luis
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Public Library of Science 2016
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4764328/
https://www.ncbi.nlm.nih.gov/pubmed/26900700
http://dx.doi.org/10.1371/journal.pone.0150098