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FBG1 Is the Final Arbitrator of A1AT-Z Degradation

Alpha-1 antitrypsin deficiency is the leading cause of childhood liver failure and one of the most common lethal genetic diseases. The disease-causing mutant A1AT-Z fails to fold correctly and accumulates in the endoplasmic reticulum (ER) of the liver, resulting in hepatic fibrosis and hepatocellula...

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Detalles Bibliográficos
Autores principales: Wen, John H., Wen, Hsiang, Gibson-Corley, Katherine N., Glenn, Kevin A.
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Public Library of Science 2015
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4546667/
https://www.ncbi.nlm.nih.gov/pubmed/26295339
http://dx.doi.org/10.1371/journal.pone.0135591