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Respiratory chain supercomplexes set the threshold for respiration defects in human mtDNA mutant cybrids.

Human molecular genetics - 1 Jul 2006

D'Aurelio Marilena, Gajewski Carl D, Lenaz Giorgio, Manfredi Giovanni

Abstract excerpt

Mitochondrial DNA (mtDNA) mutations cause heterogeneous disorders in humans. MtDNA exists in multiple copies per cell, and mutations need to accumulate beyond a critical threshold to cause disease, because coexisting wild-type mtDNA can complement the genetic defect. A better understanding of the molecular determinants of functional complementation among mtDNA molecules could help us shedding some light on the...

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PMID: 16740593
DOI: 10.1093/hmg/ddl141

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