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Respiratory mutations lead to different pleiotropic effects on OXPHOS complexes in yeast and in human cells
Author(s) -
Marsy Sophie,
Frachon Paule,
Dujardin Geneviève,
Lombès Anne,
Lemaire Claire
Publication year - 2008
Publication title -
febs letters
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.593
H-Index - 257
eISSN - 1873-3468
pISSN - 0014-5793
DOI - 10.1016/j.febslet.2008.09.016
Subject(s) - yeast , oxidative phosphorylation , saccharomyces cerevisiae , respiratory chain , biology , mutant , microbiology and biotechnology , biochemistry , respiratory system , phenotype , mutation , chemistry , mitochondrion , gene , anatomy
Pleiotropic effects in the oxidative phosphorylation pathway (OXPHOS) were investigated in yeast respiratory mutants and in cells from patients with OXPHOS genetic alterations. The main differences between yeast and human cells were (1) the site of the primary defect that was associated with pleiotropic effects, yeast complex V and human complex IV, and (2) the nature of the complex targeted by the secondary effect, yeast complex IV and human complex I. The pleiotropic effects did not correlate with the organization of OXPHOS into supercomplexes and their functional consequences appeared to be a slowing down of the respiratory chain in order to avoid either an increase in the membrane potential or the accumulation of reduced intermediary components of the respiratory chain.