title: Restoration of mitochondrial function in cells with complex I deficiency.
abstract: The mammalian mitochondrial NADH dehydrogenase (complex I) is the major entry point for the electron transport chain. It is the largest and most complicated respiratory complex consisting of at least 46 subunits, 7 of which are encoded by mitochondrial DNA (mtDNA). Deficiency in complex I function has been associated with various human diseases including neurodegenerative diseases and the aging process. To explore ways to restore mitochondrial function in complex I-deficient cells, various cell models with mutations in genes encoding subunits for complex I have been established. In this paper, we discuss various approaches to recover mitochondrial activity, the complex I activity in particular, in cultured cells.
authors.affiliation: Department of Cellular and Structural Biology, University of Texas Health Science Center at San Antonio, San Antonio, TX 78229, USA. baiy@uthscsa.edu
authors.forename: Yidong
authors.initials: Y
authors.lastname: Bai
authors.forename: Jeong Soon
authors.initials: JS
authors.lastname: Park
authors.forename: Jian-Hong
authors.initials: JH
authors.lastname: Deng
authors.forename: Youfen
authors.initials: Y
authors.lastname: Li
authors.forename: Peiqing
authors.initials: P
authors.lastname: Hu
mesh_terms: D000818:Animals; D004272:DNA, Mitochondrial / Q000235:genetics; D042967:Electron Transport Complex I / Q000172:deficiency* / Q000235:genetics / Q000378:metabolism*; D005786:Gene Expression Regulation; D006801:Humans; D008928:Mitochondria / Q000235:genetics / Q000502:physiology*; D009154:Mutation / Q000235:genetics; D021122:Protein Subunits / Q000235:genetics / Q000378:metabolism
chemical_list: D004272:DNA, Mitochondrial; D021122:Protein Subunits; D042967:Electron Transport Complex I