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P31930

- QCR1_HUMAN

UniProt

P31930 - QCR1_HUMAN

Protein

Cytochrome b-c1 complex subunit 1, mitochondrial

Gene

UQCRC1

Organism
Homo sapiens (Human)
Status
Reviewed - Annotation score: 5 out of 5- Experimental evidence at protein leveli
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    • History
      Entry version 149 (01 Oct 2014)
      Sequence version 3 (04 Apr 2006)
      Previous versions | rss
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    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 protein may mediate formation of the complex between cytochromes c and c1.

    GO - Molecular functioni

    1. metal ion binding Source: InterPro
    2. ubiquinol-cytochrome-c reductase activity Source: ProtInc

    GO - Biological processi

    1. aerobic respiration Source: ProtInc
    2. cellular metabolic process Source: Reactome
    3. hydrogen ion transmembrane transport Source: GOC
    4. mitochondrial electron transport, ubiquinol to cytochrome c Source: Ensembl
    5. oxidation-reduction process Source: ProtInc
    6. oxidative phosphorylation Source: ProtInc
    7. respiratory electron transport chain Source: Reactome
    8. response to activity Source: Ensembl
    9. response to alkaloid Source: Ensembl
    10. small molecule metabolic process Source: Reactome

    Keywords - Biological processi

    Electron transport, Respiratory chain, Transport

    Enzyme and pathway databases

    ReactomeiREACT_22393. Respiratory electron transport.
    SignaLinkiP31930.

    Protein family/group databases

    MEROPSiM16.973.

    Names & Taxonomyi

    Protein namesi
    Recommended name:
    Cytochrome b-c1 complex subunit 1, mitochondrial
    Alternative name(s):
    Complex III subunit 1
    Core protein I
    Ubiquinol-cytochrome-c reductase complex core protein 1
    Gene namesi
    Name:UQCRC1
    OrganismiHomo sapiens (Human)
    Taxonomic identifieri9606 [NCBI]
    Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresPrimatesHaplorrhiniCatarrhiniHominidaeHomo
    ProteomesiUP000005640: Chromosome 3

    Organism-specific databases

    HGNCiHGNC:12585. UQCRC1.

    Subcellular locationi

    GO - Cellular componenti

    1. mitochondrial inner membrane Source: Reactome
    2. mitochondrial respiratory chain Source: ProtInc
    3. mitochondrial respiratory chain complex III Source: Ensembl
    4. mitochondrion Source: UniProt

    Keywords - Cellular componenti

    Membrane, Mitochondrion, Mitochondrion inner membrane

    Pathology & Biotechi

    Organism-specific databases

    PharmGKBiPA37216.

    PTM / Processingi

    Molecule processing

    Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
    Transit peptidei1 – 3434Mitochondrion1 PublicationAdd
    BLAST
    Chaini35 – 480446Cytochrome b-c1 complex subunit 1, mitochondrialPRO_0000026786Add
    BLAST

    Amino acid modifications

    Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
    Modified residuei111 – 1111N6-acetyllysine1 Publication
    Modified residuei138 – 1381N6-acetyllysineBy similarity
    Modified residuei163 – 1631N6-acetyllysine; alternateBy similarity
    Modified residuei163 – 1631N6-succinyllysine; alternateBy similarity
    Modified residuei248 – 2481N6-acetyllysineBy similarity

    Keywords - PTMi

    Acetylation

    Proteomic databases

    MaxQBiP31930.
    PaxDbiP31930.
    PeptideAtlasiP31930.
    PRIDEiP31930.

    2D gel databases

    OGPiP31930.
    REPRODUCTION-2DPAGEIPI00013847.
    SWISS-2DPAGEP31930.
    UCD-2DPAGEP31930.

    PTM databases

    PhosphoSiteiP31930.

    Expressioni

    Gene expression databases

    ArrayExpressiP31930.
    BgeeiP31930.
    CleanExiHS_UQCRC1.
    GenevestigatoriP31930.

    Organism-specific databases

    HPAiCAB033782.
    HPA002815.
    HPA003525.

    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

    BioGridi113230. 33 interactions.
    IntActiP31930. 10 interactions.
    MINTiMINT-3012677.
    STRINGi9606.ENSP00000203407.

    Structurei

    3D structure databases

    ProteinModelPortaliP31930.
    SMRiP31930. Positions 35-476.
    ModBaseiSearch...
    MobiDBiSearch...

    Family & Domainsi

    Sequence similaritiesi

    Keywords - Domaini

    Transit peptide

    Phylogenomic databases

    eggNOGiCOG0612.
    HOGENOMiHOG000242450.
    HOVERGENiHBG006393.
    InParanoidiP31930.
    KOiK00414.
    OMAiFFLQGQW.
    OrthoDBiEOG74R1QJ.
    PhylomeDBiP31930.
    TreeFamiTF105032.

    Family and domain databases

    Gene3Di3.30.830.10. 2 hits.
    InterProiIPR011249. Metalloenz_LuxS/M16.
    IPR011237. Pept_M16_dom.
    IPR011765. Pept_M16_N.
    IPR007863. Peptidase_M16_C.
    [Graphical view]
    PfamiPF00675. Peptidase_M16. 1 hit.
    PF05193. Peptidase_M16_C. 1 hit.
    [Graphical view]
    SUPFAMiSSF63411. SSF63411. 2 hits.

    Sequencei

    Sequence statusi: Complete.

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

    P31930-1 [UniParc]FASTAAdd to Basket

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    MAASVVCRAA TAGAQVLLRA RRSPALLRTP ALRSTATFAQ ALQFVPETQV    50
    SLLDNGLRVA SEQSSQPTCT VGVWIDVGSR FETEKNNGAG YFLEHLAFKG 100
    TKNRPGSALE KEVESMGAHL NAYSTREHTA YYIKALSKDL PKAVELLGDI 150
    VQNCSLEDSQ IEKERDVILR EMQENDASMR DVVFNYLHAT AFQGTPLAQA 200
    VEGPSENVRK LSRADLTEYL STHYKAPRMV LAAAGGVEHQ QLLDLAQKHL 250
    GGIPWTYAED AVPTLTPCRF TGSEIRHRDD ALPFAHVAIA VEGPGWASPD 300
    NVALQVANAI IGHYDCTYGG GVHLSSPLAS GAVANKLCQS FQTFSICYAE 350
    TGLLGAHFVC DRMKIDDMMF VLQGQWMRLC TSATESEVAR GKNILRNALV 400
    SHLDGTTPVC EDIGRSLLTY GRRIPLAEWE SRIAEVDASV VREICSKYIY 450
    DQCPAVAGYG PIEQLPDYNR IRSGMFWLRF 480
    Length:480
    Mass (Da):52,646
    Last modified:April 4, 2006 - v3
    Checksum:iE76B082166CAF48F
    GO

    Natural variant

    Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
    Natural varianti215 – 2151D → H.
    Corresponds to variant rs17080284 [ dbSNP | Ensembl ].
    VAR_034581
    Natural varianti301 – 3011N → S.2 Publications
    Corresponds to variant rs144710790 [ dbSNP | Ensembl ].
    VAR_013629

    Sequence databases

    Select the link destinations:
    EMBL
    GenBank
    DDBJ
    Links Updated
    L16842 mRNA. Translation: AAA20046.1.
    D26485 mRNA. Translation: BAA05495.1.
    AK313090 mRNA. Translation: BAG35915.1.
    CH471055 Genomic DNA. Translation: EAW64898.1.
    BC009586 mRNA. Translation: AAH09586.1.
    CCDSiCCDS2774.1.
    PIRiA48043.
    RefSeqiNP_003356.2. NM_003365.2.
    UniGeneiHs.119251.

    Genome annotation databases

    EnsembliENST00000203407; ENSP00000203407; ENSG00000010256.
    GeneIDi7384.
    KEGGihsa:7384.
    UCSCiuc003cub.1. human.

    Polymorphism databases

    DMDMi92090651.

    Keywords - Coding sequence diversityi

    Polymorphism

    Cross-referencesi

    Sequence databases

    Select the link destinations:
    EMBL
    GenBank
    DDBJ
    Links Updated
    L16842 mRNA. Translation: AAA20046.1 .
    D26485 mRNA. Translation: BAA05495.1 .
    AK313090 mRNA. Translation: BAG35915.1 .
    CH471055 Genomic DNA. Translation: EAW64898.1 .
    BC009586 mRNA. Translation: AAH09586.1 .
    CCDSi CCDS2774.1.
    PIRi A48043.
    RefSeqi NP_003356.2. NM_003365.2.
    UniGenei Hs.119251.

    3D structure databases

    ProteinModelPortali P31930.
    SMRi P31930. Positions 35-476.
    ModBasei Search...
    MobiDBi Search...

    Protein-protein interaction databases

    BioGridi 113230. 33 interactions.
    IntActi P31930. 10 interactions.
    MINTi MINT-3012677.
    STRINGi 9606.ENSP00000203407.

    Chemistry

    DrugBanki DB01117. Atovaquone.

    Protein family/group databases

    MEROPSi M16.973.

    PTM databases

    PhosphoSitei P31930.

    Polymorphism databases

    DMDMi 92090651.

    2D gel databases

    OGPi P31930.
    REPRODUCTION-2DPAGE IPI00013847.
    SWISS-2DPAGE P31930.
    UCD-2DPAGE P31930.

    Proteomic databases

    MaxQBi P31930.
    PaxDbi P31930.
    PeptideAtlasi P31930.
    PRIDEi P31930.

    Protocols and materials databases

    Structural Biology Knowledgebase Search...

    Genome annotation databases

    Ensembli ENST00000203407 ; ENSP00000203407 ; ENSG00000010256 .
    GeneIDi 7384.
    KEGGi hsa:7384.
    UCSCi uc003cub.1. human.

    Organism-specific databases

    CTDi 7384.
    GeneCardsi GC03M048636.
    HGNCi HGNC:12585. UQCRC1.
    HPAi CAB033782.
    HPA002815.
    HPA003525.
    MIMi 191328. gene.
    neXtProti NX_P31930.
    PharmGKBi PA37216.
    GenAtlasi Search...

    Phylogenomic databases

    eggNOGi COG0612.
    HOGENOMi HOG000242450.
    HOVERGENi HBG006393.
    InParanoidi P31930.
    KOi K00414.
    OMAi FFLQGQW.
    OrthoDBi EOG74R1QJ.
    PhylomeDBi P31930.
    TreeFami TF105032.

    Enzyme and pathway databases

    Reactomei REACT_22393. Respiratory electron transport.
    SignaLinki P31930.

    Miscellaneous databases

    ChiTaRSi UQCRC1. human.
    GeneWikii UQCRC1.
    GenomeRNAii 7384.
    NextBioi 28912.
    PROi P31930.
    SOURCEi Search...

    Gene expression databases

    ArrayExpressi P31930.
    Bgeei P31930.
    CleanExi HS_UQCRC1.
    Genevestigatori P31930.

    Family and domain databases

    Gene3Di 3.30.830.10. 2 hits.
    InterProi IPR011249. Metalloenz_LuxS/M16.
    IPR011237. Pept_M16_dom.
    IPR011765. Pept_M16_N.
    IPR007863. Peptidase_M16_C.
    [Graphical view ]
    Pfami PF00675. Peptidase_M16. 1 hit.
    PF05193. Peptidase_M16_C. 1 hit.
    [Graphical view ]
    SUPFAMi SSF63411. SSF63411. 2 hits.
    ProtoNeti Search...

    Publicationsi

    1. "Complete coding sequence, intron/exon organization, and chromosomal location of the gene for the core I protein of human ubiquinol-cytochrome c reductase."
      Hoffman G.G., Lee S., Christiano A.M., Chung-Honet L.C., Cheng W., Katchman S., Uitto J., Greenspan D.S.
      J. Biol. Chem. 268:21113-21119(1993) [PubMed] [Europe PMC] [Abstract]
      Cited for: NUCLEOTIDE SEQUENCE [MRNA], VARIANT SER-301.
      Tissue: Placenta.
    2. "A complete cDNA sequence for core I protein subunit of human ubiquinol-cytochrome c reductase."
      Islam M.M., Tanaka M., Suzuki H., Torii K., Hattori N., Ozawa T.
      Biochem. Mol. Biol. Int. 32:797-805(1994) [PubMed] [Europe PMC] [Abstract]
      Cited for: NUCLEOTIDE SEQUENCE [MRNA].
      Tissue: Fibroblast.
    3. Erratum
      Islam M.M., Tanaka M., Suzuki H., Torii K., Hattori N., Ozawa T.
      Biochem. Mol. Biol. Int. 33:410-410(1994) [PubMed] [Europe PMC] [Abstract]
    4. Erratum
      Islam M.M., Tanaka M., Suzuki H., Torii K., Hattori N., Ozawa T.
      Biochem. Mol. Biol. Int. 33:815-815(1994) [PubMed] [Europe PMC] [Abstract]
    5. "Complete sequencing and characterization of 21,243 full-length human cDNAs."
      Ota T., Suzuki Y., Nishikawa T., Otsuki T., Sugiyama T., Irie R., Wakamatsu A., Hayashi K., Sato H., Nagai K., Kimura K., Makita H., Sekine M., Obayashi M., Nishi T., Shibahara T., Tanaka T., Ishii S.
      , Yamamoto J., Saito K., Kawai Y., Isono Y., Nakamura Y., Nagahari K., Murakami K., Yasuda T., Iwayanagi T., Wagatsuma M., Shiratori A., Sudo H., Hosoiri T., Kaku Y., Kodaira H., Kondo H., Sugawara M., Takahashi M., Kanda K., Yokoi T., Furuya T., Kikkawa E., Omura Y., Abe K., Kamihara K., Katsuta N., Sato K., Tanikawa M., Yamazaki M., Ninomiya K., Ishibashi T., Yamashita H., Murakawa K., Fujimori K., Tanai H., Kimata M., Watanabe M., Hiraoka S., Chiba Y., Ishida S., Ono Y., Takiguchi S., Watanabe S., Yosida M., Hotuta T., Kusano J., Kanehori K., Takahashi-Fujii A., Hara H., Tanase T.-O., Nomura Y., Togiya S., Komai F., Hara R., Takeuchi K., Arita M., Imose N., Musashino K., Yuuki H., Oshima A., Sasaki N., Aotsuka S., Yoshikawa Y., Matsunawa H., Ichihara T., Shiohata N., Sano S., Moriya S., Momiyama H., Satoh N., Takami S., Terashima Y., Suzuki O., Nakagawa S., Senoh A., Mizoguchi H., Goto Y., Shimizu F., Wakebe H., Hishigaki H., Watanabe T., Sugiyama A., Takemoto M., Kawakami B., Yamazaki M., Watanabe K., Kumagai A., Itakura S., Fukuzumi Y., Fujimori Y., Komiyama M., Tashiro H., Tanigami A., Fujiwara T., Ono T., Yamada K., Fujii Y., Ozaki K., Hirao M., Ohmori Y., Kawabata A., Hikiji T., Kobatake N., Inagaki H., Ikema Y., Okamoto S., Okitani R., Kawakami T., Noguchi S., Itoh T., Shigeta K., Senba T., Matsumura K., Nakajima Y., Mizuno T., Morinaga M., Sasaki M., Togashi T., Oyama M., Hata H., Watanabe M., Komatsu T., Mizushima-Sugano J., Satoh T., Shirai Y., Takahashi Y., Nakagawa K., Okumura K., Nagase T., Nomura N., Kikuchi H., Masuho Y., Yamashita R., Nakai K., Yada T., Nakamura Y., Ohara O., Isogai T., Sugano S.
      Nat. Genet. 36:40-45(2004) [PubMed] [Europe PMC] [Abstract]
      Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE MRNA].
      Tissue: Subthalamic nucleus.
    6. Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE GENOMIC DNA].
    7. "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].
      Tissue: Bone marrow.
    8. Cited for: PROTEIN SEQUENCE OF 35-45.
      Tissue: Liver.
    9. Lubec G., Vishwanath V., Chen W.-Q., Sun Y.
      Submitted (DEC-2008) to UniProtKB
      Cited for: PROTEIN SEQUENCE OF 86-99; 214-225; 229-248; 397-415; 424-442; 448-470 AND 473-479, IDENTIFICATION BY MASS SPECTROMETRY.
      Tissue: Brain, Cajal-Retzius cell and Fetal brain cortex.
    10. "Vectorial proteomics reveal targeting, phosphorylation and specific fragmentation of polymerase I and transcript release factor (PTRF) at the surface of caveolae in human adipocytes."
      Aboulaich N., Vainonen J.P., Stralfors P., Vener A.V.
      Biochem. J. 383:237-248(2004) [PubMed] [Europe PMC] [Abstract]
      Cited for: PROTEIN SEQUENCE OF 143-165; 181-209 AND 397-415.
      Tissue: Adipocyte.
    11. "Lysine acetylation targets protein complexes and co-regulates major cellular functions."
      Choudhary C., Kumar C., Gnad F., Nielsen M.L., Rehman M., Walther T.C., Olsen J.V., Mann M.
      Science 325:834-840(2009) [PubMed] [Europe PMC] [Abstract]
      Cited for: ACETYLATION [LARGE SCALE ANALYSIS] AT LYS-111, IDENTIFICATION BY MASS SPECTROMETRY [LARGE SCALE ANALYSIS].
    12. Cited for: IDENTIFICATION BY MASS SPECTROMETRY [LARGE SCALE ANALYSIS].
    13. "A mitochondrial cytochrome b mutation but no mutations of nuclearly encoded subunits in ubiquinol cytochrome c reductase (complex III) deficiency."
      Valnot I., Kassis J., Chretien D., de Lonlay P., Parfait B., Munnich A., Kachaner J., Rustin P., Roetig A.
      Hum. Genet. 104:460-466(1999) [PubMed] [Europe PMC] [Abstract]
      Cited for: VARIANT SER-301.

    Entry informationi

    Entry nameiQCR1_HUMAN
    AccessioniPrimary (citable) accession number: P31930
    Secondary accession number(s): B2R7R8, Q96DD2
    Entry historyi
    Integrated into UniProtKB/Swiss-Prot: July 1, 1993
    Last sequence update: April 4, 2006
    Last modified: October 1, 2014
    This is version 149 of the entry and version 3 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

    Does not seem to have a protease activity as it lack the zinc-binding site.Curated

    Keywords - Technical termi

    Complete proteome, Direct protein sequencing, Reference proteome

    Documents

    1. Human chromosome 3
      Human chromosome 3: 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. Peptidase families
      Classification of peptidase families and list of entries
    6. SIMILARITY comments
      Index of protein domains and families

    External Data

    Dasty 3