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Protein

Cytochrome b-c1 complex subunit 2, mitochondrial

Gene

UQCRC2

Organism
Homo sapiens (Human)
Status
Reviewed-Annotation score: -Experimental evidence at protein leveli

Functioni

This is a component of the ubiquinol-cytochrome c reductase complex (complex III or cytochrome b-c1 complex), which is part of the mitochondrial respiratory chain. The core protein 2 is required for the assembly of the complex.

Caution

Does not seem to have a protease activity as it lack the zinc-binding site.Curated

GO - Molecular functioni

  • metal ion binding Source: InterPro
  • metalloendopeptidase activity Source: GO_Central
  • protein-containing complex binding Source: Ensembl

GO - Biological processi

Keywordsi

Biological processElectron transport, Respiratory chain, Transport

Enzyme and pathway databases

ReactomeiR-HSA-611105 Respiratory electron transport
SignaLinkiP22695

Protein family/group databases

MEROPSiM16.974

Names & Taxonomyi

Protein namesi
Recommended name:
Cytochrome b-c1 complex subunit 2, mitochondrial
Alternative name(s):
Complex III subunit 2
Core protein II
Ubiquinol-cytochrome-c reductase complex core protein 2
Gene namesi
Name:UQCRC2
OrganismiHomo sapiens (Human)
Taxonomic identifieri9606 [NCBI]
Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresPrimatesHaplorrhiniCatarrhiniHominidaeHomo
Proteomesi
  • UP000005640 Componenti: Chromosome 16

Organism-specific databases

EuPathDBiHostDB:ENSG00000140740.10
HGNCiHGNC:12586 UQCRC2
MIMi191329 gene
neXtProtiNX_P22695

Subcellular locationi

Extracellular region or secreted Cytosol Plasma membrane Cytoskeleton Lysosome Endosome Peroxisome ER Golgi apparatus Nucleus Mitochondrion Manual annotation Automatic computational assertionGraphics by Christian Stolte; Source: COMPARTMENTS

Keywords - Cellular componenti

Membrane, Mitochondrion, Mitochondrion inner membrane

Pathology & Biotechi

Involvement in diseasei

Mitochondrial complex III deficiency, nuclear 5 (MC3DN5)1 Publication
The disease is caused by mutations affecting the gene represented in this entry.
Disease descriptionA disorder of the mitochondrial respiratory chain resulting in a highly variable phenotype depending on which tissues are affected. Clinical features include mitochondrial encephalopathy, psychomotor retardation, ataxia, severe failure to thrive, liver dysfunction, renal tubulopathy, muscle weakness and exercise intolerance.
See also OMIM:615160
Feature keyPosition(s)DescriptionActionsGraphical viewLength
Natural variantiVAR_069709183R → W in MC3DN5. 1 PublicationCorresponds to variant dbSNP:rs374661051EnsemblClinVar.1

Keywords - Diseasei

Disease mutation, Primary mitochondrial disease

Organism-specific databases

DisGeNETi7385
MalaCardsiUQCRC2
MIMi615160 phenotype
OpenTargetsiENSG00000140740
Orphaneti1460 Isolated complex III deficiency
PharmGKBiPA37217

Chemistry databases

DrugBankiDB08453 2-NONYL-4-HYDROXYQUINOLINE N-OXIDE
DB07778 FAMOXADONE
DB07401 METHYL (2Z)-2-(2-{[6-(2-CYANOPHENOXY)PYRIMIDIN-4-YL]OXY}PHENYL)-3-METHOXYACRYLATE
DB08330 METHYL (2Z)-3-METHOXY-2-{2-[(E)-2-PHENYLVINYL]PHENYL}ACRYLATE
DB08690 UBIQUINONE-2

Polymorphism and mutation databases

BioMutaiUQCRC2
DMDMi21903482

PTM / Processingi

Molecule processing

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Transit peptidei1 – 14MitochondrionAdd BLAST14
ChainiPRO_000002679115 – 453Cytochrome b-c1 complex subunit 2, mitochondrialAdd BLAST439

Amino acid modifications

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Modified residuei66N6-acetyllysineBy similarity1
Modified residuei199N6-acetyllysineBy similarity1
Modified residuei250N6-acetyllysineBy similarity1

Keywords - PTMi

Acetylation

Proteomic databases

EPDiP22695
MaxQBiP22695
PaxDbiP22695
PeptideAtlasiP22695
PRIDEiP22695
ProteomicsDBi54028
TopDownProteomicsiP22695

2D gel databases

REPRODUCTION-2DPAGEiIPI00305383
UCD-2DPAGEiP22695

PTM databases

CarbonylDBiP22695
GlyConnecti1166
iPTMnetiP22695
PhosphoSitePlusiP22695
SwissPalmiP22695

Expressioni

Gene expression databases

BgeeiENSG00000140740 Expressed in 235 organ(s), highest expression level in heart
CleanExiHS_UQCRC2
ExpressionAtlasiP22695 baseline and differential
GenevisibleiP22695 HS

Organism-specific databases

HPAiHPA007998
HPA019146

Interactioni

Subunit structurei

The bc1 complex contains 11 subunits: 3 respiratory subunits (cytochrome b, cytochrome c1 and Rieske/UQCRFS1), 2 core proteins (UQCRC1/QCR1 and UQCRC2/QCR2) and 6 low-molecular weight proteins (UQCRH/QCR6, UQCRB/QCR7, UQCRQ/QCR8, UQCR10/QCR9, UQCR11/QCR10 and a cleavage product of Rieske/UQCRFS1).

Binary interactionsi

WithEntry#Exp.IntActNotes
CACNA1AO005552EBI-1051424,EBI-766279

Protein-protein interaction databases

BioGridi113231, 153 interactors
ComplexPortaliCPX-560 Mitochondrial respiratory chain complex III
IntActiP22695, 37 interactors
MINTiP22695
STRINGi9606.ENSP00000268379

Structurei

Secondary structure

1453
Legend: HelixTurnBeta strandPDB Structure known for this area
Show more details

3D structure databases

ProteinModelPortaliP22695
SMRiP22695
ModBaseiSearch...
MobiDBiSearch...

Family & Domainsi

Sequence similaritiesi

Keywords - Domaini

Transit peptide

Phylogenomic databases

eggNOGiKOG2583 Eukaryota
COG0612 LUCA
GeneTreeiENSGT00550000074666
HOGENOMiHOG000046923
HOVERGENiHBG055236
InParanoidiP22695
KOiK00415
OMAiQFVQNNF
OrthoDBiEOG091G0A7X
PhylomeDBiP22695
TreeFamiTF105033

Family and domain databases

InterProiView protein in InterPro
IPR011249 Metalloenz_LuxS/M16
IPR011765 Pept_M16_N
IPR001431 Pept_M16_Zn_BS
IPR007863 Peptidase_M16_C
PfamiView protein in Pfam
PF00675 Peptidase_M16, 1 hit
PF05193 Peptidase_M16_C, 1 hit
SUPFAMiSSF63411 SSF63411, 2 hits
PROSITEiView protein in PROSITE
PS00143 INSULINASE, 1 hit

Sequence (1+)i

Sequence statusi: Complete.

Sequence processingi: The displayed sequence is further processed into a mature form.

This entry has 1 described isoform and 6 potential isoforms that are computationally mapped.Show allAlign All

P22695-1 [UniParc]FASTAAdd to basket
« Hide
        10         20         30         40         50
MKLLTRAGSF SRFYSLKVAP KVKATAAPAG APPQPQDLEF TKLPNGLVIA
60 70 80 90 100
SLENYSPVSR IGLFIKAGSR YEDFSNLGTT HLLRLTSSLT TKGASSFKIT
110 120 130 140 150
RGIEAVGGKL SVTATRENMA YTVECLRGDV DILMEFLLNV TTAPEFRRWE
160 170 180 190 200
VADLQPQLKI DKAVAFQNPQ THVIENLHAA AYRNALANPL YCPDYRIGKV
210 220 230 240 250
TSEELHYFVQ NHFTSARMAL IGLGVSHPVL KQVAEQFLNM RGGLGLSGAK
260 270 280 290 300
ANYRGGEIRE QNGDSLVHAA FVAESAVAGS AEANAFSVLQ HVLGAGPHVK
310 320 330 340 350
RGSNTTSHLH QAVAKATQQP FDVSAFNASY SDSGLFGIYT ISQATAAGDV
360 370 380 390 400
IKAAYNQVKT IAQGNLSNTD VQAAKNKLKA GYLMSVESSE CFLEEVGSQA
410 420 430 440 450
LVAGSYMPPS TVLQQIDSVA NADIINAAKK FVSGQKSMAA SGNLGHTPFV

DEL
Length:453
Mass (Da):48,443
Last modified:July 11, 2002 - v3
Checksum:iBFA655C24C07AF52
GO

Computationally mapped potential isoform sequencesi

There are 6 potential isoforms mapped to this entry.BLASTAlignShow allAdd to basket
EntryEntry nameProtein names
Gene namesLengthAnnotation
H3BRG4H3BRG4_HUMAN
Cytochrome b-c1 complex subunit 2, ...
UQCRC2
412Annotation score:
H3BP04H3BP04_HUMAN
Cytochrome b-c1 complex subunit 2, ...
UQCRC2
176Annotation score:
H3BSJ9H3BSJ9_HUMAN
Cytochrome b-c1 complex subunit 2, ...
UQCRC2
336Annotation score:
H3BUI9H3BUI9_HUMAN
Cytochrome b-c1 complex subunit 2, ...
UQCRC2 hCG_18625
132Annotation score:
H3BUE4H3BUE4_HUMAN
Cytochrome b-c1 complex subunit 2, ...
UQCRC2
116Annotation score:
A0A087WVZ4A0A087WVZ4_HUMAN
Cytochrome b-c1 complex subunit 2, ...
UQCRC2
70Annotation score:

Experimental Info

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Sequence conflicti360T → R in AAA35710 (PubMed:2547763).Curated1

Natural variant

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Natural variantiVAR_029336148R → S. Corresponds to variant dbSNP:rs2228473EnsemblClinVar.1
Natural variantiVAR_034582183R → Q1 PublicationCorresponds to variant dbSNP:rs4850EnsemblClinVar.1
Natural variantiVAR_069709183R → W in MC3DN5. 1 PublicationCorresponds to variant dbSNP:rs374661051EnsemblClinVar.1
Natural variantiVAR_036479208F → Y in a colorectal cancer sample; somatic mutation. 1 Publication1
Natural variantiVAR_034583254R → H. Corresponds to variant dbSNP:rs11863893EnsemblClinVar.1

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
J04973 mRNA Translation: AAA35710.1
AK094006 mRNA Translation: BAG52796.1
CH471249 Genomic DNA Translation: EAW50592.1
BC003136 mRNA Translation: AAH03136.1
BC000484 mRNA Translation: AAH00484.1
CCDSiCCDS10601.1
PIRiA32629
RefSeqiNP_003357.2, NM_003366.3
UniGeneiHs.528803

Genome annotation databases

EnsembliENST00000268379; ENSP00000268379; ENSG00000140740
ENST00000639558; ENSP00000491124; ENSG00000284493
GeneIDi7385
KEGGihsa:7385
UCSCiuc002djx.4 human

Keywords - Coding sequence diversityi

Polymorphism

Similar proteinsi

Cross-referencesi

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
J04973 mRNA Translation: AAA35710.1
AK094006 mRNA Translation: BAG52796.1
CH471249 Genomic DNA Translation: EAW50592.1
BC003136 mRNA Translation: AAH03136.1
BC000484 mRNA Translation: AAH00484.1
CCDSiCCDS10601.1
PIRiA32629
RefSeqiNP_003357.2, NM_003366.3
UniGeneiHs.528803

3D structure databases

Select the link destinations:
PDBei
RCSB PDBi
PDBji
Links Updated
PDB entryMethodResolution (Å)ChainPositionsPDBsum
5XTEelectron microscopy3.40K/W35-453[»]
5XTHelectron microscopy3.90AK/AW35-453[»]
5XTIelectron microscopy17.40AK/AW35-453[»]
ProteinModelPortaliP22695
SMRiP22695
ModBaseiSearch...
MobiDBiSearch...

Protein-protein interaction databases

BioGridi113231, 153 interactors
ComplexPortaliCPX-560 Mitochondrial respiratory chain complex III
IntActiP22695, 37 interactors
MINTiP22695
STRINGi9606.ENSP00000268379

Chemistry databases

DrugBankiDB08453 2-NONYL-4-HYDROXYQUINOLINE N-OXIDE
DB07778 FAMOXADONE
DB07401 METHYL (2Z)-2-(2-{[6-(2-CYANOPHENOXY)PYRIMIDIN-4-YL]OXY}PHENYL)-3-METHOXYACRYLATE
DB08330 METHYL (2Z)-3-METHOXY-2-{2-[(E)-2-PHENYLVINYL]PHENYL}ACRYLATE
DB08690 UBIQUINONE-2

Protein family/group databases

MEROPSiM16.974

PTM databases

CarbonylDBiP22695
GlyConnecti1166
iPTMnetiP22695
PhosphoSitePlusiP22695
SwissPalmiP22695

Polymorphism and mutation databases

BioMutaiUQCRC2
DMDMi21903482

2D gel databases

REPRODUCTION-2DPAGEiIPI00305383
UCD-2DPAGEiP22695

Proteomic databases

EPDiP22695
MaxQBiP22695
PaxDbiP22695
PeptideAtlasiP22695
PRIDEiP22695
ProteomicsDBi54028
TopDownProteomicsiP22695

Protocols and materials databases

DNASUi7385
Structural Biology KnowledgebaseSearch...

Genome annotation databases

EnsembliENST00000268379; ENSP00000268379; ENSG00000140740
ENST00000639558; ENSP00000491124; ENSG00000284493
GeneIDi7385
KEGGihsa:7385
UCSCiuc002djx.4 human

Organism-specific databases

CTDi7385
DisGeNETi7385
EuPathDBiHostDB:ENSG00000140740.10
GeneCardsiUQCRC2
HGNCiHGNC:12586 UQCRC2
HPAiHPA007998
HPA019146
MalaCardsiUQCRC2
MIMi191329 gene
615160 phenotype
neXtProtiNX_P22695
OpenTargetsiENSG00000140740
Orphaneti1460 Isolated complex III deficiency
PharmGKBiPA37217
GenAtlasiSearch...

Phylogenomic databases

eggNOGiKOG2583 Eukaryota
COG0612 LUCA
GeneTreeiENSGT00550000074666
HOGENOMiHOG000046923
HOVERGENiHBG055236
InParanoidiP22695
KOiK00415
OMAiQFVQNNF
OrthoDBiEOG091G0A7X
PhylomeDBiP22695
TreeFamiTF105033

Enzyme and pathway databases

ReactomeiR-HSA-611105 Respiratory electron transport
SignaLinkiP22695

Miscellaneous databases

ChiTaRSiUQCRC2 human
GeneWikiiUQCRC2
GenomeRNAii7385
PROiPR:P22695
SOURCEiSearch...

Gene expression databases

BgeeiENSG00000140740 Expressed in 235 organ(s), highest expression level in heart
CleanExiHS_UQCRC2
ExpressionAtlasiP22695 baseline and differential
GenevisibleiP22695 HS

Family and domain databases

InterProiView protein in InterPro
IPR011249 Metalloenz_LuxS/M16
IPR011765 Pept_M16_N
IPR001431 Pept_M16_Zn_BS
IPR007863 Peptidase_M16_C
PfamiView protein in Pfam
PF00675 Peptidase_M16, 1 hit
PF05193 Peptidase_M16_C, 1 hit
SUPFAMiSSF63411 SSF63411, 2 hits
PROSITEiView protein in PROSITE
PS00143 INSULINASE, 1 hit
ProtoNetiSearch...

Entry informationi

Entry nameiQCR2_HUMAN
AccessioniPrimary (citable) accession number: P22695
Secondary accession number(s): B3KSN4, Q9BQ05
Entry historyiIntegrated into UniProtKB/Swiss-Prot: August 1, 1991
Last sequence update: July 11, 2002
Last modified: November 7, 2018
This is version 189 of the entry and version 3 of the sequence. See complete history.
Entry statusiReviewed (UniProtKB/Swiss-Prot)
Annotation programChordata Protein Annotation Program
DisclaimerAny medical or genetic information present in this entry is provided for research, educational and informational purposes only. It is not in any way intended to be used as a substitute for professional medical advice, diagnosis, treatment or care.

Miscellaneousi

Keywords - Technical termi

3D-structure, Complete proteome, Direct protein sequencing, Reference proteome

Documents

  1. SIMILARITY comments
    Index of protein domains and families
  2. Human chromosome 16
    Human chromosome 16: entries, gene names and cross-references to MIM
  3. Human entries with polymorphisms or disease mutations
    List of human entries with polymorphisms or disease mutations
  4. Peptidase families
    Classification of peptidase families and list of entries
  5. Human polymorphisms and disease mutations
    Index of human polymorphisms and disease mutations
  6. MIM cross-references
    Online Mendelian Inheritance in Man (MIM) cross-references in UniProtKB/Swiss-Prot
  7. PDB cross-references
    Index of Protein Data Bank (PDB) cross-references
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