title: Mitochondrial resetting and metabolic reprogramming in induced pluripotent stem cells and mitochondrial disease modeling.
abstract: BACKGROUND
  Nuclear reprogramming with pluripotency factors enables somatic cells to gain the properties of embryonic stem cells. Mitochondrial resetting and metabolic reprogramming are suggested to be key early events in the induction of human skin fibroblasts to induced pluripotent stem cells (iPSCs).
  
  
  METHODS
  We review recent advances in the study of the molecular basis for mitochondrial resetting and metabolic reprogramming in the regulation of the formation of iPSCs. In particular, the recent progress in using iPSCs for mitochondrial disease modeling was discussed.
  
  
  CONCLUSIONS
  iPSCs rely on glycolysis rather than oxidative phosphorylation as a major supply of energy. Mitochondrial resetting and metabolic reprogramming thus play crucial roles in the process of generation of iPSCs from somatic cells.
  
  
  CONCLUSIONS
  Neurons, myocytes, and cardiomyocytes are cells containing abundant mitochondria in the human body, which can be differentiated from iPSCs or trans-differentiated from fibroblasts. Generating these cells from iPSCs derived from skin fibroblasts of patients with mitochondrial diseases or by trans-differentiation with cell-specific transcription factors will provide valuable insights into the role of mitochondrial DNA heteroplasmy in mitochondrial disease modeling and serves as a novel platform for screening of drugs to treat patients with mitochondrial diseases.
authors.affiliation: Institute of Biomedical Sciences, Mackay Medical College, New Taipei City, 252, Taiwan.
authors.forename: Yi-Chao
authors.initials: YC
authors.lastname: Hsu
authors.affiliation: Department of Medicine, Mackay Medical College, New Taipei City, 252, Taiwan.
authors.forename: Chien-Tsun
authors.initials: CT
authors.lastname: Chen
authors.affiliation: Institute of Biomedical Sciences, Mackay Medical College, New Taipei City, 252, Taiwan; Department of Medicine, Mackay Medical College, New Taipei City, 252, Taiwan. Electronic address: yhweibabi@gmail.com.
authors.forename: Yau-Huei
authors.initials: YH
authors.lastname: Wei
mesh_terms: D000818:Animals; D065150:Cellular Reprogramming; D006801:Humans; D057026:Induced Pluripotent Stem Cells / Q000378:metabolism* / Q000473:pathology; D008928:Mitochondria / Q000235:genetics / Q000378:metabolism* / Q000473:pathology; D028361:Mitochondrial Diseases / Q000235:genetics / Q000378:metabolism* / Q000473:pathology; D008954:Models, Biological
keywords: Glycolysis; Induced pluripotent stem cells; Metabolic reprogramming; Mitochondrial disease