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Autophagy master regulator TFEB induces clearance of toxic SERPINA1/α-1-antitrypsin polymers

Deficiency of SERPINA1/AAT [serpin peptidase inhibitor, clade A (α-1 antiproteinase, antitrypsin), member 1/α 1-antitrypsin] results in polymerization and aggregation of mutant SERPINA1 molecules in the endoplasmic reticulum of hepatocytes, triggering liver injury. SERPINA1 deficiency is the most co...

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Bibliografski detalji
Glavni autori: Pastore, Nunzia, Ballabio, Andrea, Brunetti-Pierri, Nicola
Format: Artigo
Jezik:Inglês
Izdano: Landes Bioscience 2013
Teme:
Online pristup:https://ncbi.nlm.nih.gov/pmc/articles/PMC3722318/
https://ncbi.nlm.nih.gov/pubmed/23584152
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.4161/auto.24469
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