Synthesis of cytochrome c oxidase subunit 1 is translationally downregulated in the absence of functional F1F0-ATP synthase.

doi:10.1016/j.bbamcr.2009.09.002

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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...

Topics

Aerobiosis
Carbonyl Cyanide m-Chlorophenyl Hydrazone
Electron Transport Complex IV
Fibroblasts
Gene Expression Regulation, Enzymologic
Gene Expression Regulation, Fungal
H(+)-K(+)-Exchanging ATPase
Humans
Mitochondrial Encephalomyopathies

Methodology Data sources Editorial policy Privacy About Contact Helena Bioinformatics

Models, Biological

Mutation

Oligomycins

Proton-Translocating ATPases

Saccharomyces cerevisiae

Saccharomyces cerevisiae Proteins

Uncoupling Agents

Source

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Identifiers

PMID: 19735676
DOI: 10.1016/j.bbamcr.2009.09.002
PMCID: PMC2764804

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