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Protein

Cytochrome b-c1 complex subunit 7

Gene

UQCRB

Organism
Homo sapiens (Human)
Status
Reviewed-Annotation score: Annotation score: 5 out of 5-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. This component is involved in redox-linked proton pumping.

GO - Biological processi

  • aerobic respiration Source: ProtInc
  • cellular metabolic process Source: Reactome
  • mitochondrial electron transport, ubiquinol to cytochrome c Source: InterPro
  • oxidation-reduction process Source: ProtInc
  • oxidative phosphorylation Source: ProtInc
  • respiratory electron transport chain Source: Reactome
  • small molecule metabolic process Source: Reactome
Complete GO annotation...

Keywords - Biological processi

Electron transport, Respiratory chain, Transport

Enzyme and pathway databases

ReactomeiREACT_22393. Respiratory electron transport.

Names & Taxonomyi

Protein namesi
Recommended name:
Cytochrome b-c1 complex subunit 7
Alternative name(s):
Complex III subunit 7
Complex III subunit VII
QP-C
Ubiquinol-cytochrome c reductase complex 14 kDa protein
Gene namesi
Name:UQCRB
Synonyms:UQBP
OrganismiHomo sapiens (Human)
Taxonomic identifieri9606 [NCBI]
Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresPrimatesHaplorrhiniCatarrhiniHominidaeHomo
ProteomesiUP000005640 Componenti: Chromosome 8

Organism-specific databases

HGNCiHGNC:12582. UQCRB.

Subcellular locationi

GO - Cellular componenti

Complete GO annotation...

Keywords - Cellular componenti

Membrane, Mitochondrion, Mitochondrion inner membrane

Pathology & Biotechi

Involvement in diseasei

Mitochondrial complex III deficiency, nuclear 3 (MC3DN3)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:615158

Organism-specific databases

MIMi615158. phenotype.
Orphaneti1460. Isolated CoQ-cytochrome C reductase deficiency.
PharmGKBiPA37213.

Polymorphism and mutation databases

BioMutaiUQCRB.
DMDMi136717.

PTM / Processingi

Molecule processing

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Initiator methioninei1 – 11RemovedBy similarity
Chaini2 – 111110Cytochrome b-c1 complex subunit 7PRO_0000193524Add
BLAST

Amino acid modifications

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Modified residuei2 – 21N-acetylalanineBy similarity
Modified residuei12 – 121N6-acetyllysine; alternateBy similarity
Modified residuei12 – 121N6-succinyllysine; alternateBy similarity
Modified residuei19 – 191N6-acetyllysineBy similarity
Modified residuei78 – 781N6-acetyllysine; alternateBy similarity
Modified residuei78 – 781N6-succinyllysine; alternateBy similarity
Modified residuei83 – 831N6-acetyllysineBy similarity
Modified residuei96 – 961N6-acetyllysineBy similarity

Keywords - PTMi

Acetylation

Proteomic databases

MaxQBiP14927.
PaxDbiP14927.
PRIDEiP14927.

2D gel databases

UCD-2DPAGEP14927.

PTM databases

PhosphoSiteiP14927.

Expressioni

Gene expression databases

BgeeiP14927.
CleanExiHS_UQCRB.
ExpressionAtlasiP14927. baseline and differential.
GenevisibleiP14927. HS.

Organism-specific databases

HPAiHPA043060.

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
ACCSQ96QU63EBI-743128,EBI-743387
MAGEA4Q1RN333EBI-743128,EBI-10194128

Protein-protein interaction databases

BioGridi113227. 36 interactions.
IntActiP14927. 7 interactions.
MINTiMINT-1471523.
STRINGi9606.ENSP00000287022.

Structurei

3D structure databases

ProteinModelPortaliP14927.
SMRiP14927. Positions 13-111.
ModBaseiSearch...
MobiDBiSearch...

Family & Domainsi

Sequence similaritiesi

Belongs to the UQCRB/QCR7 family.Curated

Phylogenomic databases

eggNOGiNOG235867.
GeneTreeiENSGT00390000012916.
HOGENOMiHOG000188221.
HOVERGENiHBG002984.
InParanoidiP14927.
KOiK00417.
OMAiPENVYND.
OrthoDBiEOG7VDXR7.
PhylomeDBiP14927.
TreeFamiTF105035.

Family and domain databases

Gene3Di1.10.1090.10. 1 hit.
InterProiIPR003197. QCR7.
[Graphical view]
PANTHERiPTHR12022. PTHR12022. 1 hit.
PfamiPF02271. UCR_14kD. 1 hit.
[Graphical view]
PIRSFiPIRSF000022. Bc1_14K. 1 hit.
ProDomiPD008153. Cyt_bd_ubiquinol_oxidase_14kDa. 1 hit.
[Graphical view] [Entries sharing at least one domain]
SUPFAMiSSF81524. SSF81524. 1 hit.

Sequences (2)i

Sequence statusi: Complete.

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

This entry describes 2 isoformsi produced by alternative splicing. AlignAdd to basket

Isoform 1 (identifier: P14927-1) [UniParc]FASTAAdd to basket

This isoform has been chosen as the 'canonical' sequence. All positional information in this entry refers to it. This is also the sequence that appears in the downloadable versions of the entry.

« Hide

        10         20         30         40         50
MAGKQAVSAS GKWLDGIRKW YYNAAGFNKL GLMRDDTIYE DEDVKEAIRR
60 70 80 90 100
LPENLYNDRM FRIKRALDLN LKHQILPKEQ WTKYEEENFY LEPYLKEVIR
110
ERKEREEWAK K
Length:111
Mass (Da):13,530
Last modified:January 23, 2007 - v2
Checksum:iE61629D06DFA55B7
GO
Isoform 2 (identifier: P14927-2) [UniParc]FASTAAdd to basket

The sequence of this isoform differs from the canonical sequence as follows:
     87-111: ENFYLEPYLKEVIRERKEREEWAKK → VFAVPALHSA...QRKISTLNRI

Note: No experimental confirmation available.
Show »
Length:140
Mass (Da):16,267
Checksum:i478C935E0C90E188
GO

Experimental Info

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Sequence conflicti6 – 61A → G in AAA60236 (PubMed:2543413).Curated

Natural variant

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Natural varianti30 – 301L → P.
Corresponds to variant rs35895613 [ dbSNP | Ensembl ].
VAR_052443

Alternative sequence

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Alternative sequencei87 – 11125ENFYL…EWAKK → VFAVPALHSASYLDEKISPL SVPPDPKKSFCEANSHPLNC IETRQRKISTLNRI in isoform 2. 1 PublicationVSP_045601Add
BLAST

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
X13585 mRNA. Translation: CAA31926.1.
M22348 mRNA. Translation: AAA60238.1.
CR542176 mRNA. Translation: CAG46973.1.
CR542196 mRNA. Translation: CAG46993.1.
M26730
, M35761, M26706, M26707 Genomic DNA. Translation: AAA60235.1.
M26700 mRNA. Translation: AAA60236.1.
M26701 Genomic DNA. Translation: AAA60237.1.
AP003465 Genomic DNA. No translation available.
CH471060 Genomic DNA. Translation: EAW91749.1.
BC005230 mRNA. Translation: AAH05230.1.
BU535281 mRNA. No translation available.
M37387 Genomic DNA. Translation: AAA60361.1. Sequence problems.
CCDSiCCDS59107.1. [P14927-2]
CCDS6269.1. [P14927-1]
PIRiA32450.
RefSeqiNP_001186904.1. NM_001199975.2.
NP_001241681.1. NM_001254752.1. [P14927-2]
NP_006285.1. NM_006294.4. [P14927-1]
UniGeneiHs.131255.

Genome annotation databases

EnsembliENST00000287022; ENSP00000287022; ENSG00000156467.
ENST00000518406; ENSP00000430494; ENSG00000156467. [P14927-2]
GeneIDi7381.
KEGGihsa:7381.
UCSCiuc003yhq.4. human. [P14927-1]

Keywords - Coding sequence diversityi

Alternative splicing, Polymorphism

Cross-referencesi

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
X13585 mRNA. Translation: CAA31926.1.
M22348 mRNA. Translation: AAA60238.1.
CR542176 mRNA. Translation: CAG46973.1.
CR542196 mRNA. Translation: CAG46993.1.
M26730
, M35761, M26706, M26707 Genomic DNA. Translation: AAA60235.1.
M26700 mRNA. Translation: AAA60236.1.
M26701 Genomic DNA. Translation: AAA60237.1.
AP003465 Genomic DNA. No translation available.
CH471060 Genomic DNA. Translation: EAW91749.1.
BC005230 mRNA. Translation: AAH05230.1.
BU535281 mRNA. No translation available.
M37387 Genomic DNA. Translation: AAA60361.1. Sequence problems.
CCDSiCCDS59107.1. [P14927-2]
CCDS6269.1. [P14927-1]
PIRiA32450.
RefSeqiNP_001186904.1. NM_001199975.2.
NP_001241681.1. NM_001254752.1. [P14927-2]
NP_006285.1. NM_006294.4. [P14927-1]
UniGeneiHs.131255.

3D structure databases

ProteinModelPortaliP14927.
SMRiP14927. Positions 13-111.
ModBaseiSearch...
MobiDBiSearch...

Protein-protein interaction databases

BioGridi113227. 36 interactions.
IntActiP14927. 7 interactions.
MINTiMINT-1471523.
STRINGi9606.ENSP00000287022.

Chemistry

ChEMBLiCHEMBL1671612.

PTM databases

PhosphoSiteiP14927.

Polymorphism and mutation databases

BioMutaiUQCRB.
DMDMi136717.

2D gel databases

UCD-2DPAGEP14927.

Proteomic databases

MaxQBiP14927.
PaxDbiP14927.
PRIDEiP14927.

Protocols and materials databases

DNASUi7381.
Structural Biology KnowledgebaseSearch...

Genome annotation databases

EnsembliENST00000287022; ENSP00000287022; ENSG00000156467.
ENST00000518406; ENSP00000430494; ENSG00000156467. [P14927-2]
GeneIDi7381.
KEGGihsa:7381.
UCSCiuc003yhq.4. human. [P14927-1]

Organism-specific databases

CTDi7381.
GeneCardsiGC08M097311.
HGNCiHGNC:12582. UQCRB.
HPAiHPA043060.
MIMi191330. gene.
615158. phenotype.
neXtProtiNX_P14927.
Orphaneti1460. Isolated CoQ-cytochrome C reductase deficiency.
PharmGKBiPA37213.
GenAtlasiSearch...

Phylogenomic databases

eggNOGiNOG235867.
GeneTreeiENSGT00390000012916.
HOGENOMiHOG000188221.
HOVERGENiHBG002984.
InParanoidiP14927.
KOiK00417.
OMAiPENVYND.
OrthoDBiEOG7VDXR7.
PhylomeDBiP14927.
TreeFamiTF105035.

Enzyme and pathway databases

ReactomeiREACT_22393. Respiratory electron transport.

Miscellaneous databases

GeneWikiiUQCRB.
GenomeRNAii7381.
NextBioi28904.
PROiP14927.
SOURCEiSearch...

Gene expression databases

BgeeiP14927.
CleanExiHS_UQCRB.
ExpressionAtlasiP14927. baseline and differential.
GenevisibleiP14927. HS.

Family and domain databases

Gene3Di1.10.1090.10. 1 hit.
InterProiIPR003197. QCR7.
[Graphical view]
PANTHERiPTHR12022. PTHR12022. 1 hit.
PfamiPF02271. UCR_14kD. 1 hit.
[Graphical view]
PIRSFiPIRSF000022. Bc1_14K. 1 hit.
ProDomiPD008153. Cyt_bd_ubiquinol_oxidase_14kDa. 1 hit.
[Graphical view] [Entries sharing at least one domain]
SUPFAMiSSF81524. SSF81524. 1 hit.
ProtoNetiSearch...

Publicationsi

« Hide 'large scale' publications
  1. "Cloning and sequencing of a cDNA for human mitochondrial ubiquinone-binding protein of complex III."
    Suzuki H., Yoshitaka H., Toda H., Nishikimi M., Ozawa T.
    Biochem. Biophys. Res. Commun. 156:987-994(1988) [PubMed] [Europe PMC] [Abstract]
    Cited for: NUCLEOTIDE SEQUENCE [MRNA] (ISOFORM 1).
    Tissue: Fibroblast.
  2. "Isolation of a single nuclear gene encoding human ubiquinone-binding protein in complex III of mitochondrial respiratory chain."
    Suzuki H., Hosokawa Y., Toda H., Nishikimi M., Ozawa T.
    Biochem. Biophys. Res. Commun. 161:371-378(1989) [PubMed] [Europe PMC] [Abstract]
    Cited for: NUCLEOTIDE SEQUENCE [GENOMIC DNA / MRNA] (ISOFORM 1).
  3. "Cloning of human full open reading frames in Gateway(TM) system entry vector (pDONR201)."
    Ebert L., Schick M., Neubert P., Schatten R., Henze S., Korn B.
    Submitted (JUN-2004) to the EMBL/GenBank/DDBJ databases
    Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE MRNA] (ISOFORM 1).
  4. "DNA sequence and analysis of human chromosome 8."
    Nusbaum C., Mikkelsen T.S., Zody M.C., Asakawa S., Taudien S., Garber M., Kodira C.D., Schueler M.G., Shimizu A., Whittaker C.A., Chang J.L., Cuomo C.A., Dewar K., FitzGerald M.G., Yang X., Allen N.R., Anderson S., Asakawa T.
    , Blechschmidt K., Bloom T., Borowsky M.L., Butler J., Cook A., Corum B., DeArellano K., DeCaprio D., Dooley K.T., Dorris L. III, Engels R., Gloeckner G., Hafez N., Hagopian D.S., Hall J.L., Ishikawa S.K., Jaffe D.B., Kamat A., Kudoh J., Lehmann R., Lokitsang T., Macdonald P., Major J.E., Matthews C.D., Mauceli E., Menzel U., Mihalev A.H., Minoshima S., Murayama Y., Naylor J.W., Nicol R., Nguyen C., O'Leary S.B., O'Neill K., Parker S.C.J., Polley A., Raymond C.K., Reichwald K., Rodriguez J., Sasaki T., Schilhabel M., Siddiqui R., Smith C.L., Sneddon T.P., Talamas J.A., Tenzin P., Topham K., Venkataraman V., Wen G., Yamazaki S., Young S.K., Zeng Q., Zimmer A.R., Rosenthal A., Birren B.W., Platzer M., Shimizu N., Lander E.S.
    Nature 439:331-335(2006) [PubMed] [Europe PMC] [Abstract]
    Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE GENOMIC DNA].
  5. Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE GENOMIC DNA].
  6. "The status, quality, and expansion of the NIH full-length cDNA project: the Mammalian Gene Collection (MGC)."
    The MGC Project Team
    Genome Res. 14:2121-2127(2004) [PubMed] [Europe PMC] [Abstract]
    Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE MRNA] (ISOFORMS 1 AND 2).
    Tissue: Urinary bladder.
  7. "Common protein-binding sites in the 5'-flanking regions of human genes for cytochrome c1 and ubiquinone-binding protein."
    Suzuki H., Hosokawa Y., Toda H., Nishikimi M., Ozawa T.
    J. Biol. Chem. 265:8159-8163(1990) [PubMed] [Europe PMC] [Abstract]
    Cited for: NUCLEOTIDE SEQUENCE [GENOMIC DNA] OF 1-6.
  8. "A deletion in the human QP-C gene causes a complex III deficiency resulting in hypoglycaemia and lactic acidosis."
    Haut S., Brivet M., Touati G., Rustin P., Lebon S., Garcia-Cazorla A., Saudubray J.-M., Boutron A., Legrand A., Slama A.
    Hum. Genet. 113:118-122(2003) [PubMed] [Europe PMC] [Abstract]
    Cited for: INVOLVEMENT IN MC3DN3.
  9. Cited for: IDENTIFICATION BY MASS SPECTROMETRY [LARGE SCALE ANALYSIS].
  10. "An enzyme assisted RP-RPLC approach for in-depth analysis of human liver phosphoproteome."
    Bian Y., Song C., Cheng K., Dong M., Wang F., Huang J., Sun D., Wang L., Ye M., Zou H.
    J. Proteomics 96:253-262(2014) [PubMed] [Europe PMC] [Abstract]
    Cited for: IDENTIFICATION BY MASS SPECTROMETRY [LARGE SCALE ANALYSIS].
    Tissue: Liver.

Entry informationi

Entry nameiQCR7_HUMAN
AccessioniPrimary (citable) accession number: P14927
Secondary accession number(s): E5RJU0, Q6FGD1
Entry historyi
Integrated into UniProtKB/Swiss-Prot: April 1, 1990
Last sequence update: January 23, 2007
Last modified: July 22, 2015
This is version 156 of the entry and version 2 of the sequence. [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

Caution

Was originally thought to be the ubiquinone-binding protein (QP-C).Curated

Keywords - Technical termi

Complete proteome, Reference proteome

Documents

  1. Human chromosome 8
    Human chromosome 8: 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. SIMILARITY comments
    Index of protein domains and families

External Data

Dasty 3

Similar proteinsi

Links to similar proteins from the UniProt Reference Clusters (UniRef) at 100%, 90% and 50% sequence identity:
100%UniRef100 combines identical sequences and sub-fragments with 11 or more residues from any organism into Uniref entry.
90%UniRef90 is built by clustering UniRef100 sequences that have at least 90% sequence identity to, and 80% overlap with, the longest sequence (a.k.a seed sequence).
50%UniRef50 is built by clustering UniRef90 seed sequences that have at least 50% sequence identity to, and 80% overlap with, the longest sequence in the cluster.