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P14927

- QCR7_HUMAN

UniProt

P14927 - QCR7_HUMAN

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Protein

Cytochrome b-c1 complex subunit 7

Gene
UQCRB, UQBP
Organism
Homo sapiens (Human)
Status
Reviewed - 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

  1. aerobic respiration Source: ProtInc
  2. cellular metabolic process Source: Reactome
  3. mitochondrial electron transport, ubiquinol to cytochrome c Source: InterPro
  4. oxidation-reduction process Source: ProtInc
  5. oxidative phosphorylation Source: ProtInc
  6. respiratory electron transport chain Source: Reactome
  7. 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: Chromosome 8

Organism-specific databases

HGNCiHGNC:12582. UQCRB.

Subcellular locationi

GO - Cellular componenti

  1. mitochondrial inner membrane Source: Reactome
  2. mitochondrial respiratory chain Source: ProtInc
  3. mitochondrial respiratory chain complex III Source: InterPro
Complete GO annotation...

Keywords - Cellular componenti

Membrane, Mitochondrion, Mitochondrion inner membrane

Pathology & Biotechi

Involvement in diseasei

Mitochondrial complex III deficiency, nuclear 3 (MC3DN3) [MIM:615158]: A 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.
Note: The disease is caused by mutations affecting the gene represented in this entry.1 Publication

Organism-specific databases

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

PTM / Processingi

Molecule processing

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

Amino acid modifications

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

Keywords - PTMi

Acetylation

Proteomic databases

MaxQBiP14927.
PaxDbiP14927.
PRIDEiP14927.

2D gel databases

UCD-2DPAGEP14927.

PTM databases

PhosphoSiteiP14927.

Expressioni

Gene expression databases

ArrayExpressiP14927.
BgeeiP14927.
CleanExiHS_UQCRB.
GenevestigatoriP14927.

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).

Protein-protein interaction databases

BioGridi113227. 25 interactions.
IntActiP14927. 3 interactions.
MINTiMINT-1471523.
STRINGi9606.ENSP00000287022.

Structurei

3D structure databases

ProteinModelPortaliP14927.
SMRiP14927. Positions 13-111.

Family & Domainsi

Sequence similaritiesi

Belongs to the UQCRB/QCR7 family.

Phylogenomic databases

eggNOGiNOG235867.
HOGENOMiHOG000188221.
HOVERGENiHBG002984.
InParanoidiP14927.
KOiK00417.
OMAiQWVKYEE.
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. Align

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

MAGKQAVSAS GKWLDGIRKW YYNAAGFNKL GLMRDDTIYE DEDVKEAIRR    50
LPENLYNDRM FRIKRALDLN LKHQILPKEQ WTKYEEENFY LEPYLKEVIR 100
ERKEREEWAK K 111
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

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. VSP_045601Add
BLAST

Sequence conflict

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Sequence conflicti6 – 61A → G in AAA60236. 1 Publication

Sequence databases

Select the link destinations:
EMBL
GenBank
DDBJ
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. [P14927-1]
ENST00000518406; ENSP00000430494; ENSG00000156467. [P14927-2]
GeneIDi7381.
KEGGihsa:7381.
UCSCiuc003yhq.4. human. [P14927-1]

Polymorphism databases

DMDMi136717.

Keywords - Coding sequence diversityi

Alternative splicing, Polymorphism

Cross-referencesi

Sequence databases

Select the link destinations:
EMBL
GenBank
DDBJ
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.
CCDSi CCDS59107.1. [P14927-2 ]
CCDS6269.1. [P14927-1 ]
PIRi A32450.
RefSeqi NP_001186904.1. NM_001199975.2.
NP_001241681.1. NM_001254752.1. [P14927-2 ]
NP_006285.1. NM_006294.4. [P14927-1 ]
UniGenei Hs.131255.

3D structure databases

ProteinModelPortali P14927.
SMRi P14927. Positions 13-111.
ModBasei Search...
MobiDBi Search...

Protein-protein interaction databases

BioGridi 113227. 25 interactions.
IntActi P14927. 3 interactions.
MINTi MINT-1471523.
STRINGi 9606.ENSP00000287022.

Chemistry

ChEMBLi CHEMBL1671612.

PTM databases

PhosphoSitei P14927.

Polymorphism databases

DMDMi 136717.

2D gel databases

UCD-2DPAGE P14927.

Proteomic databases

MaxQBi P14927.
PaxDbi P14927.
PRIDEi P14927.

Protocols and materials databases

DNASUi 7381.
Structural Biology Knowledgebase Search...

Genome annotation databases

Ensembli ENST00000287022 ; ENSP00000287022 ; ENSG00000156467 . [P14927-1 ]
ENST00000518406 ; ENSP00000430494 ; ENSG00000156467 . [P14927-2 ]
GeneIDi 7381.
KEGGi hsa:7381.
UCSCi uc003yhq.4. human. [P14927-1 ]

Organism-specific databases

CTDi 7381.
GeneCardsi GC08M097311.
HGNCi HGNC:12582. UQCRB.
HPAi HPA043060.
MIMi 191330. gene.
615158. phenotype.
neXtProti NX_P14927.
Orphaneti 1460. Isolated CoQ-cytochrome C reductase deficiency.
PharmGKBi PA37213.
GenAtlasi Search...

Phylogenomic databases

eggNOGi NOG235867.
HOGENOMi HOG000188221.
HOVERGENi HBG002984.
InParanoidi P14927.
KOi K00417.
OMAi QWVKYEE.
OrthoDBi EOG7VDXR7.
PhylomeDBi P14927.
TreeFami TF105035.

Enzyme and pathway databases

Reactomei REACT_22393. Respiratory electron transport.

Miscellaneous databases

GeneWikii UQCRB.
GenomeRNAii 7381.
NextBioi 28904.
PROi P14927.
SOURCEi Search...

Gene expression databases

ArrayExpressi P14927.
Bgeei P14927.
CleanExi HS_UQCRB.
Genevestigatori P14927.

Family and domain databases

Gene3Di 1.10.1090.10. 1 hit.
InterProi IPR003197. QCR7.
[Graphical view ]
PANTHERi PTHR12022. PTHR12022. 1 hit.
Pfami PF02271. UCR_14kD. 1 hit.
[Graphical view ]
PIRSFi PIRSF000022. Bc1_14K. 1 hit.
ProDomi PD008153. Cyt_bd_ubiquinol_oxidase_14kDa. 1 hit.
[Graphical view ] [Entries sharing at least one domain ]
SUPFAMi SSF81524. SSF81524. 1 hit.
ProtoNeti Search...

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].

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: September 3, 2014
This is version 147 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

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

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