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P14927 (QCR7_HUMAN) Reviewed, UniProtKB/Swiss-Prot

Last modified July 9, 2014. Version 146. Feed History...

Clusters with 100%, 90%, 50% identity | Documents (5) | Third-party data text xml rdf/xml gff fasta
to top of pageNames·Attributes·General annotation·Ontologies·Alt products·Sequence annotation·Sequences·References·Cross-refs·Entry info·DocumentsCustomize order

Names and origin

Protein namesRecommended 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 names
Name:UQCRB
Synonyms:UQBP
OrganismHomo sapiens (Human) [Reference proteome]
Taxonomic identifier9606 [NCBI]
Taxonomic lineageEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresPrimatesHaplorrhiniCatarrhiniHominidaeHomo

Protein attributes

Sequence length111 AA.
Sequence statusComplete.
Sequence processingThe displayed sequence is further processed into a mature form.
Protein existenceEvidence at protein level

General annotation (Comments)

Function

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.

Subunit structure

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).

Subcellular location

Mitochondrion inner membrane.

Involvement in disease

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. Ref.8

Sequence similarities

Belongs to the UQCRB/QCR7 family.

Caution

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

Alternative products

This entry describes 2 isoforms produced by alternative splicing. [Align] [Select]
Isoform 1 (identifier: P14927-1)

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.
Isoform 2 (identifier: P14927-2)

The sequence of this isoform differs from the canonical sequence as follows:
     87-111: ENFYLEPYLKEVIRERKEREEWAKK → VFAVPALHSA...QRKISTLNRI
Note: No experimental confirmation available.

Sequence annotation (Features)

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifier

Molecule processing

Initiator methionine11Removed By similarity
Chain2 – 111110Cytochrome b-c1 complex subunit 7
PRO_0000193524

Amino acid modifications

Modified residue21N-acetylalanine By similarity
Modified residue121N6-acetyllysine; alternate By similarity
Modified residue121N6-succinyllysine; alternate By similarity
Modified residue191N6-acetyllysine By similarity
Modified residue781N6-acetyllysine; alternate By similarity
Modified residue781N6-succinyllysine; alternate By similarity
Modified residue831N6-acetyllysine By similarity
Modified residue961N6-acetyllysine By similarity

Natural variations

Alternative sequence87 – 11125ENFYL…EWAKK → VFAVPALHSASYLDEKISPL SVPPDPKKSFCEANSHPLNC IETRQRKISTLNRI in isoform 2.
VSP_045601
Natural variant301L → P.
Corresponds to variant rs35895613 [ dbSNP | Ensembl ].
VAR_052443

Experimental info

Sequence conflict61A → G in AAA60236. Ref.2

Sequences

Sequence LengthMass (Da)Tools
Isoform 1 [UniParc].

Last modified January 23, 2007. Version 2.
Checksum: E61629D06DFA55B7

FASTA11113,530
        10         20         30         40         50         60 
MAGKQAVSAS GKWLDGIRKW YYNAAGFNKL GLMRDDTIYE DEDVKEAIRR LPENLYNDRM 

        70         80         90        100        110 
FRIKRALDLN LKHQILPKEQ WTKYEEENFY LEPYLKEVIR ERKEREEWAK K 

« Hide

Isoform 2 [UniParc].

Checksum: 478C935E0C90E188
Show »

FASTA14016,267

References

« Hide 'large scale' references
[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. expand/collapse author list , 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]Mural R.J., Istrail S., Sutton G.G., Florea L., Halpern A.L., Mobarry C.M., Lippert R., Walenz B., Shatkay H., Dew I., Miller J.R., Flanigan M.J., Edwards N.J., Bolanos R., Fasulo D., Halldorsson B.V., Hannenhalli S., Turner R. expand/collapse author list , Yooseph S., Lu F., Nusskern D.R., Shue B.C., Zheng X.H., Zhong F., Delcher A.L., Huson D.H., Kravitz S.A., Mouchard L., Reinert K., Remington K.A., Clark A.G., Waterman M.S., Eichler E.E., Adams M.D., Hunkapiller M.W., Myers E.W., Venter J.C.
Submitted (JUL-2005) to the EMBL/GenBank/DDBJ databases
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]"Initial characterization of the human central proteome."
Burkard T.R., Planyavsky M., Kaupe I., Breitwieser F.P., Buerckstuemmer T., Bennett K.L., Superti-Furga G., Colinge J.
BMC Syst. Biol. 5:17-17(2011) [PubMed] [Europe PMC] [Abstract]
Cited for: IDENTIFICATION BY MASS SPECTROMETRY [LARGE SCALE ANALYSIS].
+Additional computationally mapped references.

Cross-references

Sequence databases

EMBL
GenBank
DDBJ
X13585 mRNA. Translation: CAA31926.1.
M22348 mRNA. Translation: AAA60238.1.
CR542176 mRNA. Translation: CAG46973.1.
CR542196 mRNA. Translation: CAG46993.1.
M26730 expand/collapse EMBL AC list , 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.
CCDSCCDS59107.1. [P14927-2]
CCDS6269.1. [P14927-1]
PIRA32450.
RefSeqNP_001186904.1. NM_001199975.2.
NP_001241681.1. NM_001254752.1. [P14927-2]
NP_006285.1. NM_006294.4. [P14927-1]
UniGeneHs.131255.

3D structure databases

ProteinModelPortalP14927.
SMRP14927. Positions 13-111.
ModBaseSearch...
MobiDBSearch...

Protein-protein interaction databases

BioGrid113227. 24 interactions.
IntActP14927. 3 interactions.
MINTMINT-1471523.
STRING9606.ENSP00000287022.

Chemistry

ChEMBLCHEMBL1671612.

PTM databases

PhosphoSiteP14927.

Polymorphism databases

DMDM136717.

2D gel databases

UCD-2DPAGEP14927.

Proteomic databases

MaxQBP14927.
PaxDbP14927.
PRIDEP14927.

Protocols and materials databases

DNASU7381.
StructuralBiologyKnowledgebaseSearch...

Genome annotation databases

EnsemblENST00000287022; ENSP00000287022; ENSG00000156467. [P14927-1]
ENST00000518406; ENSP00000430494; ENSG00000156467. [P14927-2]
GeneID7381.
KEGGhsa:7381.
UCSCuc003yhq.4. human. [P14927-1]

Organism-specific databases

CTD7381.
GeneCardsGC08M097311.
HGNCHGNC:12582. UQCRB.
HPAHPA043060.
MIM191330. gene.
615158. phenotype.
neXtProtNX_P14927.
Orphanet1460. Isolated CoQ-cytochrome C reductase deficiency.
PharmGKBPA37213.
GenAtlasSearch...

Phylogenomic databases

eggNOGNOG235867.
HOGENOMHOG000188221.
HOVERGENHBG002984.
InParanoidP14927.
KOK00417.
OMAQWVKYEE.
OrthoDBEOG7VDXR7.
PhylomeDBP14927.
TreeFamTF105035.

Enzyme and pathway databases

ReactomeREACT_111217. Metabolism.

Gene expression databases

ArrayExpressP14927.
BgeeP14927.
CleanExHS_UQCRB.
GenevestigatorP14927.

Family and domain databases

Gene3D1.10.1090.10. 1 hit.
InterProIPR003197. QCR7.
[Graphical view]
PANTHERPTHR12022. PTHR12022. 1 hit.
PfamPF02271. UCR_14kD. 1 hit.
[Graphical view]
PIRSFPIRSF000022. Bc1_14K. 1 hit.
ProDomPD008153. Cyt_bd_ubiquinol_oxidase_14kDa. 1 hit.
[Graphical view] [Entries sharing at least one domain]
SUPFAMSSF81524. SSF81524. 1 hit.
ProtoNetSearch...

Other

GeneWikiUQCRB.
GenomeRNAi7381.
NextBio28904.
PROP14927.
SOURCESearch...

Entry information

Entry nameQCR7_HUMAN
AccessionPrimary (citable) accession number: P14927
Secondary accession number(s): E5RJU0, Q6FGD1
Entry history
Integrated into UniProtKB/Swiss-Prot: April 1, 1990
Last sequence update: January 23, 2007
Last modified: July 9, 2014
This is version 146 of the entry and version 2 of the sequence. [Complete history]
Entry statusReviewed (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.

Relevant documents

SIMILARITY comments

Index of protein domains and families

MIM cross-references

Online Mendelian Inheritance in Man (MIM) cross-references in UniProtKB/Swiss-Prot

Human polymorphisms and disease mutations

Index of human polymorphisms and disease mutations

Human entries with polymorphisms or disease mutations

List of human entries with polymorphisms or disease mutations

Human chromosome 8

Human chromosome 8: entries, gene names and cross-references to MIM