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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

GO - Biological processi

  • aerobic respiration Source: ProtInc
  • oxidative phosphorylation Source: ProtInc
  • protein processing involved in protein targeting to mitochondrion Source: GO_Central

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 CoQ-cytochrome C reductase 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
TopDownProteomicsiP22695

2D gel databases

REPRODUCTION-2DPAGEIPI00305383
UCD-2DPAGEP22695

PTM databases

CarbonylDBiP22695
iPTMnetiP22695
PhosphoSitePlusiP22695
SwissPalmiP22695

Expressioni

Gene expression databases

BgeeiENSG00000140740
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, 150 interactors
IntActiP22695, 25 interactors
MINTiP22695
STRINGi9606.ENSP00000268379

Structurei

Secondary structure

1453
Legend: HelixTurnBeta strandPDB Structure known for this area
Show more details
Feature keyPosition(s)DescriptionActionsGraphical viewLength
Beta strandi48 – 51Combined sources4
Beta strandi57 – 66Combined sources10
Helixi69 – 71Combined sources3
Helixi79 – 83Combined sources5
Helixi85 – 88Combined sources4
Beta strandi91 – 93Combined sources3
Helixi96 – 106Combined sources11
Beta strandi109 – 114Combined sources6
Beta strandi119 – 126Combined sources8
Helixi127 – 129Combined sources3
Helixi130 – 142Combined sources13
Helixi148 – 154Combined sources7
Helixi157 – 165Combined sources9
Helixi169 – 181Combined sources13
Beta strandi182 – 184Combined sources3
Helixi185 – 187Combined sources3
Helixi194 – 196Combined sources3
Turni197 – 199Combined sources3
Helixi202 – 212Combined sources11
Turni215 – 217Combined sources3
Beta strandi218 – 225Combined sources8
Helixi227 – 233Combined sources7
Helixi234 – 238Combined sources5
Beta strandi257 – 259Combined sources3
Beta strandi265 – 275Combined sources11
Beta strandi278 – 280Combined sources3
Helixi281 – 292Combined sources12
Helixi308 – 314Combined sources7
Beta strandi321 – 328Combined sources8
Beta strandi334 – 343Combined sources10
Turni344 – 346Combined sources3
Helixi347 – 362Combined sources16
Helixi368 – 387Combined sources20
Helixi389 – 402Combined sources14
Helixi409 – 418Combined sources10
Helixi421 – 433Combined sources13
Beta strandi437 – 440Combined sources4

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...

Family & Domainsi

Sequence similaritiesi

Keywords - Domaini

Transit peptide

Phylogenomic databases

eggNOGiKOG2583 Eukaryota
COG0612 LUCA
GeneTreeiENSGT00550000074666
HOGENOMiHOG000046923
HOVERGENiHBG055236
InParanoidiP22695
KOiK00415
OMAiVECLRDD
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, 3 hits
PROSITEiView protein in PROSITE
PS00143 INSULINASE, 1 hit

Sequencei

Sequence statusi: Complete.

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

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

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

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: May 23, 2018
This is version 184 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. Human chromosome 16
    Human chromosome 16: entries, gene names and cross-references to MIM
  2. Human entries with polymorphisms or disease mutations
    List of human entries with polymorphisms or disease mutations
  3. Human polymorphisms and disease mutations
    Index of human polymorphisms and disease mutations
  4. MIM cross-references
    Online Mendelian Inheritance in Man (MIM) cross-references in UniProtKB/Swiss-Prot
  5. PDB cross-references
    Index of Protein Data Bank (PDB) cross-references
  6. Peptidase families
    Classification of peptidase families and list of entries
  7. SIMILARITY comments
    Index of protein domains and families

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