Article
Mitochondrial complex I deficiency: from organelle dysfunction to clinical disease.
Brain : a journal of neurology - 1 Apr 2009
Distelmaier Felix, Koopman Werner J H, van den Heuvel Lambertus P, Rodenburg Richard J, Mayatepek Ertan, Willems Peter H G M, Smeitink Jan A M
Abstract excerpt
Mitochondria are essential for cellular bioenergetics by way of energy production in the form of ATP through the process of oxidative phosphorylation. This crucial task is executed by five multi-protein complexes of which mitochondrial NADH:ubiquinone oxidoreductase or complex I is the largest and most complicated one. During recent years, mutations in nuclear genes encoding structural subunits of complex I have...
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