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Details on Person UniProt:P31930 UQCRC1

Class:IdReferenceGeneProduct:66890
_chainChangeLogtransit peptide:1-34 added on Fri February 6 2015;chain:35-480 added on Fri February 6 2015
_displayNameUniProt:P31930 UQCRC1
_timestamp2024-11-03 19:58:10
chaintransit peptide:1-34
chain:35-480
checksumE76B082166CAF48F
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 (By similarity). The 2 core subunits UQCRC1/QCR1 and UQCRC2/QCR2 are homologous to the 2 mitochondrial-processing peptidase (MPP) subunits beta-MPP and alpha-MPP respectively, and they seem to have preserved their MPP processing properties (By similarity). May be involved in the in situ processing of UQCRFS1 into the mature Rieske protein and its mitochondrial targeting sequence (MTS)/subunit 9 when incorporated into complex III (Probable). Seems to play an important role in the maintenance of proper mitochondrial function in nigral dopaminergic neurons (PubMed:33141179).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). Interacts with UQCC6 (PubMed:32161263). Interacts with STMP1 (By similarity).INTERACTION Expressed in brain, including substantia nigra, striatum, cortex and cerebellum, and in spinal cord, heart, kidney, liver and muscle.DISEASE The protein represented in this entry is involved in disease pathogenesis.SIMILARITY Belongs to the peptidase M16 family. UQCRC1/QCR1 subfamily.
descriptionrecommendedName: Cytochrome b-c1 complex subunit 1, mitochondrial alternativeName: Complex III subunit 1 alternativeName: Core protein I alternativeName: Ubiquinol-cytochrome-c reductase complex core protein 1
geneNameUQCRC1
identifierP31930
isSequenceChangedFALSE
keyword3D-structure
Acetylation
Direct protein sequencing
Disease variant
Electron transport
Membrane
Mitochondrion
Mitochondrion inner membrane
Neuropathy
Parkinsonism
Phosphoprotein
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
nameUQCRC1
referenceDatabase[ReferenceDatabase:2] UniProt
referenceGene[ReferenceDNASequence:8993848] ENSEMBL:ENSG00000010256 UQCRC1 [Homo sapiens]
secondaryIdentifierQCR1_HUMAN
B2R7R8
Q96DD2
sequenceLength480
species[Species:48887] Homo sapiens
(referenceEntity)[EntityWithAccessionedSequence:164284] UQCRC1 [mitochondrial inner membrane] [Homo sapiens]
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