Article
Synthesis of cytochrome c oxidase subunit 1 is translationally downregulated in the absence of functional F1F0-ATP synthase.
Biochimica et biophysica acta - 1 Nov 2009
Soto Ileana C, Fontanesi Flavia, Valledor Melvys, Horn Darryl, Singh Rajiv, Barrientos Antoni
Abstract excerpt
The mitochondrial F(1)F(0)-ATP synthase or ATPase is a key enzyme for aerobic energy production in eukaryotic cells. Mutations in ATPase structural and assembly genes are the primary cause of severe human encephalomyopathies, frequently associated with a pleiotropic decrease in cytochrome c oxidase (COX) activity. We have studied the structural and functional constraints underlying the COX defect using...
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