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ATP8B1 Deficiency Results in Elevated Mitochondrial Phosphatidylethanolamine Levels and Increased Mitochondrial Oxidative Phosphorylation in Human Hepatoma Cells
ATP8B1 is a phospholipid flippase that is deficient in patients with progressive familial intrahepatic cholestasis type 1 (PFIC1). PFIC1 patients suffer from severe liver disease but also present with dyslipidemia, including low plasma cholesterol, of yet unknown etiology. Here we show that ATP8B1 k...
Autores principales: | Gómez-Mellado, Valentina E., Chang, Jung-Chin, Ho-Mok, Kam S., Bernardino Morcillo, Carmen, Kersten, Remco H. J., Oude Elferink, Ronald P. J., Verhoeven, Arthur J., Paulusma, Coen C. |
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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/PMC9604224/ https://www.ncbi.nlm.nih.gov/pubmed/36293199 http://dx.doi.org/10.3390/ijms232012344 |
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