Article
Loss of mitochondrial DNA-encoded protein ND1 results in disruption of complex I biogenesis during early stages of assembly.
FASEB journal : official publication of the Federation of American Societies for Experimental Biology - 1 Jun 2016
Lim Sze Chern, Hroudová Jana, Van Bergen Nicole J, Lopez Sanchez M Isabel G, Trounce Ian A, McKenzie Matthew
Abstract excerpt
Mitochondrial complex I (NADH:ubiquinone oxidoreductase) must be assembled precisely from 45 protein subunits for it to function correctly. One of its mitochondrial DNA (mtDNA) encoded subunits, ND1, is incorporated during the early stages of complex I assembly. However, little is known about how mutations in ND1 affect this assembly process. We found that in human 143B cybrid cells carrying a homoplasmic MT-ND1...
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