The nuoM arg368his mutation in NADH:ubiquinone oxidoreductase from Rhodobacter capsulatus: a model for the human nd4-11778 mtDNA mutation associated with Leber's hereditary optic neuropathy.

Abstract excerpt

Mutation at position 11778 in the nd4 gene of the human mitochondrial complex I is associated with Leber's hereditary optic neuropathy. Type I NADH:ubiquinone oxidoreductase of Rhodobacter capsulatus displays similar properties to complex I of the mitochondrial respiratory chain. The NUOM subunit of the bacterial enzyme is homologous to the ND4 subunit. Disruption of the nuoM gene led to a bacterial mutant...

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