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Restoration of Mitochondrial Function in Cells with Complex I Deficiency
Author(s) -
BAI YIDONG,
PARK JEONG SOON,
DENG JIANHONG,
LI YOUFEN,
HU PEIQING
Publication year - 2005
Publication title -
annals of the new york academy of sciences
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.712
H-Index - 248
eISSN - 1749-6632
pISSN - 0077-8923
DOI - 10.1196/annals.1338.003
Subject(s) - mitochondrial dna , mitochondrion , point mutation , function (biology) , electron transport complex i , biology , microbiology and biotechnology , mitochondrial respiratory chain , gene , mitochondrial disease , respiratory chain , mutation , genetics
A bstract : 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.