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Details on Person UniProt:P07919 UQCRH

Class:IdReferenceGeneProduct:66896
_chainChangeLogtransit peptide:1-13 added on Sat February 7 2015;chain:14-91 added on Sat February 7 2015
_displayNameUniProt:P07919 UQCRH
_timestamp2024-11-03 19:38:25
chaintransit peptide:1-13
chain:14-91
checksum499B5D069E738F4C
commentFUNCTION Component of the ubiquinol-cytochrome c oxidoreductase, a multisubunit transmembrane complex that is part of the mitochondrial electron transport chain which drives oxidative phosphorylation. The respiratory chain contains 3 multisubunit complexes succinate dehydrogenase (complex II, CII), ubiquinol-cytochrome c oxidoreductase (cytochrome b-c1 complex, complex III, CIII) and cytochrome c oxidase (complex IV, CIV), that cooperate to transfer electrons derived from NADH and succinate to molecular oxygen, creating an electrochemical gradient over the inner membrane that drives transmembrane transport and the ATP synthase. The cytochrome b-c1 complex catalyzes electron transfer from ubiquinol to cytochrome c, linking this redox reaction to translocation of protons across the mitochondrial inner membrane, with protons being carried across the membrane as hydrogens on the quinol. In the process called Q cycle, 2 protons are consumed from the matrix, 4 protons are released into the intermembrane space and 2 electrons are passed to cytochrome c.SUBUNIT Component of the ubiquinol-cytochrome c oxidoreductase (cytochrome b-c1 complex, complex III, CIII), a multisubunit enzyme composed of 11 subunits. The complex is composed of 3 respiratory subunits cytochrome b, cytochrome c1 and Rieske protein UQCRFS1, 2 core protein subunits UQCRC1/QCR1 and UQCRC2/QCR2, and 6 low-molecular weight protein subunits UQCRH/QCR6, UQCRB/QCR7, UQCRQ/QCR8, UQCR10/QCR9, UQCR11/QCR10 and subunit 9, the cleavage product of Rieske protein UQCRFS1 (By similarity). The complex exists as an obligatory dimer and forms supercomplexes (SCs) in the inner mitochondrial membrane with NADH-ubiquinone oxidoreductase (complex I, CI) and cytochrome c oxidase (complex IV, CIV), resulting in different assemblies (supercomplex SCI(1)III(2)IV(1) and megacomplex MCI(2)III(2)IV(2)) (PubMed:28844695).INTERACTION The disease may be caused by variants affecting the gene represented in this entry.SIMILARITY Belongs to the UQCRH/QCR6 family.
descriptionrecommendedName: Cytochrome b-c1 complex subunit 6, mitochondrial alternativeName: Complex III subunit 6 alternativeName: Complex III subunit VIII alternativeName: Cytochrome c1 non-heme 11 kDa protein alternativeName: Mitochondrial hinge protein alternativeName: Ubiquinol-cytochrome c reductase complex 11 kDa protein
geneNameUQCRH
identifierP07919
isSequenceChangedFALSE
keyword3D-structure
Acetylation
Disulfide bond
Electron transport
Membrane
Mitochondrion
Mitochondrion inner membrane
Primary mitochondrial disease
Proteomics identification
Reference proteome
Respiratory chain
Transit peptide
Transport
modified[InstanceEdit:9836292] Weiser, Joel, 2023-05-25
[InstanceEdit:9852000] Weiser, Joel, 2023-11-03
[InstanceEdit:9926675] Weiser, Joel, 2024-11-03
nameUQCRH
referenceDatabase[ReferenceDatabase:2] UniProt
referenceGene[ReferenceDNASequence:8990351] ENSEMBL:ENSG00000173660 UQCRH [Homo sapiens]
secondaryIdentifierQCR6_HUMAN
B2R4V9
D3DQ18
Q5TDF6
Q6LDB8
Q9BQ91
sequenceLength91
species[Species:48887] Homo sapiens
(referenceEntity)[EntityWithAccessionedSequence:164286] UQCRH [mitochondrial inner membrane] [Homo sapiens]
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