Mitochondrial Respiratory Defect Causes Dysfunctional Lactate Turnover via AMP-activated Protein Kinase Activation in Human-induced Pluripotent Stem Cell-derived Hepatocytes

A defective mitochondrial respiratory chain complex (DMRC) causes various metabolic disorders in humans. However, the pathophysiology of DMRC in the liver remains unclear. To understand DMRC pathophysiology in vitro, DMRC-induced pluripotent stem cells were generated from dermal fibroblasts of a DMR...

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Veröffentlicht in:	The Journal of biological chemistry 2015-12, Vol.290 (49), p.29493-29505
Hauptverfasser:	Im, Ilkyun, Jang, Mi-jin, Park, Seung Ju, Lee, Sang-Hee, Choi, Jin-Ho, Yoo, Han-Wook, Kim, Seyun, Han, Yong-Mahn
Format:	Artikel
Sprache:	eng
Schlagworte:	Acidosis, Lactic - metabolism AMP-Activated Protein Kinases - metabolism Cell Biology Cell Differentiation DNA, Mitochondrial - metabolism Electron Transport Enzyme Activation Fibroblasts - metabolism Glycogen - metabolism hepatocyte Hepatocytes - cytology Hepatocytes - metabolism Humans induced pluripotent stem cell (iPS cell) (iPSC) Induced Pluripotent Stem Cells - cytology Infant Lactic Acid - metabolism lactic acidosis liver Liver - metabolism Male Microscopy, Electron, Transmission Mitochondria - metabolism mitochondrial disease Mitochondrial Diseases - metabolism Mutation NADH Dehydrogenase - genetics Point Mutation
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