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UQCRFS1 produces N-terminal polypeptides, which remain bound to holocomplex III. We show that, in normal conditions, these UQCRFS1 fragments are rapidly removed, but when TTC19 is absent they accumulate within complex III, causing its structural and functional impairment.
UQCRFS1/RISP knockdown in breast tumor cell line led to decreased mitochondrial membrane potential as well as a decrease in matrigel invasion.
UQCRFS1 gene appears to be involved in development of more aggressive phenotype of breast cancer.
Rieske iron-sulfur protein is required for superoxide generation at Complex III.
Mitochondrial respiratory complexes of the electron transport chain (CI and CIV) and supercomplexes were decreased in the RISP-deficient cells.
A 3' UTR modification of the mitochondrial rieske iron sulfur protein in mice produces a specific skin pigmentation phenotype.
This is the first direct demonstration that histidine is the redox-linked protonation site of the Rieske iron-sulfur protein subunit of cytochrome bc1.
Three hydrogen-bond mutant Rieske clusters all have decreased cluster reduction potentials and increased cluster pK values consistent with the removal of a hydrogen-bonding dipole, which preferentially stabilizes the most negatively charged state.
in the normal catalytic cycle of the bc(1) complex, the [2Fe-2S] cluster may function as a proton-exiting gate
Component of the ubiquinol-cytochrome c reductase complex (complex III or cytochrome b-c1 complex), which is a respiratory chain that generates an electrochemical potential coupled to ATP synthesis.
ubiquinol-cytochrome c reductase, Rieske iron-sulfur polypeptide 1
, cytochrome b-c1 complex subunit Rieske, mitochondrial
, OXPHOS complex III iron-sulfur subunit
, Rieske Fe-S protein
, complex III subunit 5
, rieske iron-sulfur protein
, ubiquinol-cytochrome c reductase iron-sulfur subunit
, Rieske iron-sulfur protein
, cytochrome b-c1 complex subunit 5
, liver regeneration-related protein LRRGT00195
, Rieske iron-sulfur polypeptide 1
, ubiquinol-cytochrome c reductase, Rieske iron-sulfur polypeptide-like 1