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P14927

- QCR7_HUMAN

UniProt

P14927 - QCR7_HUMAN

Protein

Cytochrome b-c1 complex subunit 7

Gene

UQCRB

Organism
Homo sapiens (Human)
Status
Reviewed - Annotation score: 5 out of 5- Experimental evidence at protein leveli
  1. 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

    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

    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.1 Publication
    Note: The disease is caused by mutations affecting the gene represented in this entry.

    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 – 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

    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.
    ModBaseiSearch...
    MobiDBiSearch...

    Family & Domainsi

    Sequence similaritiesi

    Belongs to the UQCRB/QCR7 family.Curated

    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

    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:
    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

    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: October 1, 2014
    This is version 148 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