Biochimica et Biophysica Acta (BBA) - Molecular Basis of Disease
Volume 1822, Issue 7, July 2012, Pages 1114-1124
Adaptation of respiratory chain biogenesis to cytochrome c oxidase deficiency caused by SURF1 gene mutations
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Highlights
► SURF1 mutations are frequent cause of severe COX deficiency. ► Altered energy provision leads to increase of respiratory chain complexes I, III, and V. ► Adaptive changes in mitochondrial biogenesis are due to posttranscriptional events. ► All residual COX is incorporated into I–III2–IV1 supercomplex.
Abbreviations
OXPHOS
oxidative phosphorylation
COX
cytochrome c oxidase
LSCOX
Leigh syndrome on the basis of cytochrome c oxidase deficiency
cI, cII, cIII, cIV
respiratory chain complexes I–IV
cV
mitochondrial ATP synthase
Keywords
Mitochondrial disorder
SURF1 gene
Leigh syndrome
Gene expression
Oxidative phosphorylation
Cytochrome c oxidase
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