Article
Mitochondrial ATP synthase disorders: molecular mechanisms and the quest for curative therapeutic approaches.
Biochimica et biophysica acta - 1 Jan 2009
Kucharczyk Roza, Zick Michael, Bietenhader Maïlis, Rak Malgorzata, Couplan Elodie, Blondel Marc, Caubet Stéphane-Duvezin, di Rago Jean-Paul
Abstract excerpt
In mammals, the majority of cellular ATP is produced by the mitochondrial F1F(O)-ATP synthase through an elaborate catalytic mechanism. While most subunits of this enzymatic complex are encoded by the nuclear genome, a few essential components are encoded in the mitochondrial genome. The biogenesis of this multi-subunit enzyme is a sophisticated multi-step process that is regulated on levels of transcription,...
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