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In Vitro Rescue of the Bile Acid Transport Function of ABCB11 Variants by CFTR Potentiators
ABCB11 is responsible for biliary bile acid secretion at the canalicular membrane of hepatocytes. Variations in the ABCB11 gene cause a spectrum of rare liver diseases. The most severe form is progressive familial intrahepatic cholestasis type 2 (PFIC2). Current medical treatments have limited effic...
Autores principales: | , , , , , , , , , , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
MDPI
2022
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9502978/ https://www.ncbi.nlm.nih.gov/pubmed/36142670 http://dx.doi.org/10.3390/ijms231810758 |