UniProt:P31930 UQCRC1

chain
  • transit peptide:1-34
  • chain:35-480
checksum E76B082166CAF48F
comment
  • FUNCTION 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.
crossReference
databaseName UniProt
dbId 66890
description
  • recommendedName: 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
displayName UniProt:P31930 UQCRC1
geneName
  • UQCRC1
identifier P31930
isSequenceChanged false
keyword
  • 3D-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:9926675] Weiser, Joel, 2024-11-03
moleculeType Protein
name
  • UQCRC1
otherIdentifier
  • 11743979_a_at
  • 11747855_a_at
  • 11761061_at
  • 16953568
  • 201903_PM_at
  • 201903_at
  • 2673510
  • 2673511
  • 2673512
  • 2673513
  • 2673514
  • 2673515
  • 2673516
  • 2673517
  • 2673518
  • 2673519
  • 2673520
  • 2673521
  • 2673522
  • 2673523
  • 2673524
  • 2673525
  • 2673526
  • 2673527
  • 2673528
  • 2673529
  • 2673530
  • 2673531
  • 2673532
  • 2673533
  • 2673534
  • 2673535
  • 2673536
  • 283_at
  • 7384
  • 8087100
  • A_23_P132675
  • GE57651
  • GO:0005515
  • GO:0005739
  • GO:0005743
  • GO:0006091
  • GO:0006119
  • GO:0006122
  • GO:0008121
  • GO:0009060
  • GO:0014823
  • GO:0016020
  • GO:0016491
  • GO:0031625
  • GO:0043279
  • GO:0044877
  • GO:0045275
  • GO:0045333
  • GO:0046872
  • GO:0055085
  • GO:0098803
  • GO:1902600
  • HMNXSV003052550
  • ILMN_1671191
  • L16842_at
  • PH_hs_0027224
  • TC03001384.hg
  • g4507840_3p_at
physicalEntity
referenceDatabase [ReferenceDatabase:2] UniProt
referenceGene
referenceTranscript
schemaClass ReferenceGeneProduct
secondaryIdentifier
  • QCR1_HUMAN
  • B2R7R8
  • Q96DD2
sequenceLength 480
species [Species:48887] Homo sapiens
stId uniprot:P31930
url http://purl.uniprot.org/uniprot/P31930

Referrals

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