Article
The interplay between SUCLA2, SUCLG2, and mitochondrial DNA depletion.
Biochimica et biophysica acta - 1 May 2011
Miller Chaya, Wang Liya, Ostergaard Elsebet, Dan Phyllis, Saada Ann
Abstract excerpt
SUCLA2-related mitochondrial DNA (mtDNA) depletion syndrome is a result of mutations in the β subunit of the ADP-dependent isoform of the Krebs cycle succinyl-CoA synthase (SCS). The mechanism of tissue specificity and mtDNA depletion is elusive but complementation by the GDP-dependent isoform encoded by SUCLG2, and the association with mitochondrial nucleoside diphosphate kinase (NDPK), is a plausible link. We...
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