UniProt:P22695 UQCRC2

chain
  • transit peptide:1-14
  • chain:15-453
checksum BFA655C24C07AF52
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).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 RAB5IF (PubMed:31536960). Interacts with STMP1 (By similarity).INTERACTION The disease is caused by variants affecting the gene represented in this entry.SIMILARITY Belongs to the peptidase M16 family. UQCRC2/QCR2 subfamily.
crossReference
databaseName UniProt
dbId 66892
description
  • recommendedName: Cytochrome b-c1 complex subunit 2, mitochondrial alternativeName: Complex III subunit 2 alternativeName: Core protein II alternativeName: Ubiquinol-cytochrome-c reductase complex core protein 2
displayName UniProt:P22695 UQCRC2
geneName
  • UQCRC2
identifier P22695
isSequenceChanged false
keyword
  • 3D-structure
  • Acetylation
  • Direct protein sequencing
  • Disease variant
  • Electron transport
  • Membrane
  • Mitochondrion
  • Mitochondrion inner membrane
  • Primary mitochondrial disease
  • Proteomics identification
  • Reference proteome
  • Respiratory chain
  • Transit peptide
  • Transport
modified [InstanceEdit:9926675] Weiser, Joel, 2024-11-03
moleculeType Protein
name
  • UQCRC2
otherIdentifier
  • 11744059_a_at
  • 11744060_s_at
  • 16816739
  • 200883_PM_at
  • 200883_at
  • 212600_PM_s_at
  • 212600_s_at
  • 239465_PM_at
  • 239465_at
  • 241755_PM_at
  • 241755_at
  • 3652228
  • 3652230
  • 3652231
  • 3652233
  • 3652234
  • 3652235
  • 3652236
  • 3652237
  • 3652238
  • 3652239
  • 3652240
  • 3652241
  • 3652242
  • 3652243
  • 3652244
  • 3652245
  • 3652246
  • 3652248
  • 3652249
  • 3652250
  • 3652252
  • 3652253
  • 3652255
  • 3652256
  • 3652257
  • 3652258
  • 3652260
  • 3652261
  • 3652262
  • 40854_at
  • 7385
  • 7993872
  • A_14_P124516
  • A_23_P118002
  • GE57520
  • GE86494
  • GO:0004222
  • GO:0005515
  • GO:0005654
  • GO:0005739
  • GO:0005743
  • GO:0006091
  • GO:0006119
  • GO:0006122
  • GO:0006508
  • GO:0009060
  • GO:0016020
  • GO:0045275
  • GO:0045333
  • GO:0046872
  • HMNXSV003055371
  • Hs.173554.1.A1_3p_a_at
  • Hs.265591.1.A1_3p_at
  • ILMN_1718853
  • J04973_at
  • PH_hs_0010402
  • PH_hs_0040405
  • TC16000239.hg
physicalEntity
referenceDatabase [ReferenceDatabase:2] UniProt
referenceGene
referenceTranscript
schemaClass ReferenceGeneProduct
secondaryIdentifier
  • QCR2_HUMAN
  • B3KSN4
  • Q9BQ05
sequenceLength 453
species [Species:48887] Homo sapiens
stId uniprot:P22695
url http://purl.uniprot.org/uniprot/P22695
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