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Hoffmann, R. A wiki for the life sciences where authorship matters. Nature Genetics (2008)
 
 
 

Respiratory complex III is required to maintain complex I in mammalian mitochondria.

A puzzling observation in patients with oxidative phosphorylation (OXPHOS) deficiencies is the presence of combined enzyme complex defects associated with a genetic alteration in only one protein-coding gene. In particular, mutations in the mtDNA encoded cytochrome b gene are associated either with combined complex I+III deficiency or with only complex III deficiency. We have reproduced the combined complex I+III defect in mouse and human cultured cell models harboring cytochrome b mutations. In both, complex III assembly is impeded and causes a severe reduction in the amount of complex I, not observed when complex III activity was pharmacologically inhibited. Metabolic labeling in mouse cells revealed that complex I was assembled, although its stability was severely hampered. Conversely, complex III stability was not influenced by the absence of complex I. This structural dependence among complexes I and III was confirmed in a muscle biopsy of a patient harboring a nonsense cytochrome b mutation.[1]

References

  1. Respiratory complex III is required to maintain complex I in mammalian mitochondria. Acín-Pérez, R., Bayona-Bafaluy, M.P., Fernández-Silva, P., Moreno-Loshuertos, R., Pérez-Martos, A., Bruno, C., Moraes, C.T., Enríquez, J.A. Mol. Cell (2004) [Pubmed]
 
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