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P22695

- QCR2_HUMAN

UniProt

P22695 - QCR2_HUMAN

Protein

Cytochrome b-c1 complex subunit 2, mitochondrial

Gene

UQCRC2

Organism
Homo sapiens (Human)
Status
Reviewed - Annotation score: 5 out of 5- Experimental evidence at protein leveli
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    • History
      Entry version 148 (01 Oct 2014)
      Sequence version 3 (11 Jul 2002)
      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. The core protein 2 is required for the assembly of the complex.

    GO - Molecular functioni

    1. metal ion binding Source: InterPro
    2. metalloendopeptidase activity Source: InterPro
    3. protein binding Source: IntAct

    GO - Biological processi

    1. aerobic respiration Source: ProtInc
    2. cellular metabolic process Source: Reactome
    3. oxidative phosphorylation Source: ProtInc
    4. respiratory electron transport chain Source: Reactome
    5. small molecule metabolic process Source: Reactome

    Keywords - Biological processi

    Electron transport, Respiratory chain, Transport

    Enzyme and pathway databases

    ReactomeiREACT_22393. Respiratory electron transport.
    SignaLinkiP22695.

    Protein family/group databases

    MEROPSiM16.974.

    Names & Taxonomyi

    Protein namesi
    Recommended name:
    Cytochrome b-c1 complex subunit 2, mitochondrial
    Alternative name(s):
    Complex III subunit 2
    Core protein II
    Ubiquinol-cytochrome-c reductase complex core protein 2
    Gene namesi
    Name:UQCRC2
    OrganismiHomo sapiens (Human)
    Taxonomic identifieri9606 [NCBI]
    Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresPrimatesHaplorrhiniCatarrhiniHominidaeHomo
    ProteomesiUP000005640: Chromosome 16

    Organism-specific databases

    HGNCiHGNC:12586. UQCRC2.

    Subcellular locationi

    GO - Cellular componenti

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

    Keywords - Cellular componenti

    Membrane, Mitochondrion, Mitochondrion inner membrane

    Pathology & Biotechi

    Involvement in diseasei

    Mitochondrial complex III deficiency, nuclear 5 (MC3DN5) [MIM:615160]: 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.
    Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
    Natural varianti183 – 1831R → W in MC3DN5. 1 Publication
    VAR_069709

    Keywords - Diseasei

    Disease mutation

    Organism-specific databases

    MIMi615160. phenotype.
    Orphaneti1460. Isolated CoQ-cytochrome C reductase deficiency.
    PharmGKBiPA37217.

    PTM / Processingi

    Molecule processing

    Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
    Transit peptidei1 – 1414MitochondrionAdd
    BLAST
    Chaini15 – 453439Cytochrome b-c1 complex subunit 2, mitochondrialPRO_0000026791Add
    BLAST

    Amino acid modifications

    Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
    Modified residuei66 – 661N6-acetyllysineBy similarity
    Modified residuei199 – 1991N6-acetyllysineBy similarity
    Modified residuei250 – 2501N6-acetyllysineBy similarity

    Keywords - PTMi

    Acetylation

    Proteomic databases

    MaxQBiP22695.
    PaxDbiP22695.
    PeptideAtlasiP22695.
    PRIDEiP22695.

    2D gel databases

    REPRODUCTION-2DPAGEIPI00305383.
    UCD-2DPAGEP22695.

    PTM databases

    PhosphoSiteiP22695.

    Expressioni

    Gene expression databases

    ArrayExpressiP22695.
    BgeeiP22695.
    CleanExiHS_UQCRC2.
    GenevestigatoriP22695.

    Organism-specific databases

    HPAiHPA007998.
    HPA019146.

    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
    CACNA1AO005552EBI-1051424,EBI-766279

    Protein-protein interaction databases

    BioGridi113231. 29 interactions.
    IntActiP22695. 9 interactions.
    MINTiMINT-2801885.
    STRINGi9606.ENSP00000268379.

    Structurei

    3D structure databases

    ProteinModelPortaliP22695.
    SMRiP22695. Positions 32-453.
    ModBaseiSearch...
    MobiDBiSearch...

    Family & Domainsi

    Sequence similaritiesi

    Keywords - Domaini

    Transit peptide

    Phylogenomic databases

    eggNOGiCOG0612.
    HOGENOMiHOG000046923.
    HOVERGENiHBG055236.
    InParanoidiP22695.
    KOiK00415.
    OMAiQFVQNNF.
    OrthoDBiEOG79CXZF.
    PhylomeDBiP22695.
    TreeFamiTF105033.

    Family and domain databases

    Gene3Di3.30.830.10. 2 hits.
    InterProiIPR011249. Metalloenz_LuxS/M16.
    IPR011237. Pept_M16_dom.
    IPR011765. Pept_M16_N.
    IPR001431. Pept_M16_Zn_BS.
    IPR007863. Peptidase_M16_C.
    [Graphical view]
    PfamiPF00675. Peptidase_M16. 1 hit.
    PF05193. Peptidase_M16_C. 1 hit.
    [Graphical view]
    SUPFAMiSSF63411. SSF63411. 3 hits.
    PROSITEiPS00143. INSULINASE. 1 hit.
    [Graphical view]

    Sequencei

    Sequence statusi: Complete.

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

    P22695-1 [UniParc]FASTAAdd to Basket

    « Hide

    MKLLTRAGSF SRFYSLKVAP KVKATAAPAG APPQPQDLEF TKLPNGLVIA    50
    SLENYSPVSR IGLFIKAGSR YEDFSNLGTT HLLRLTSSLT TKGASSFKIT 100
    RGIEAVGGKL SVTATRENMA YTVECLRGDV DILMEFLLNV TTAPEFRRWE 150
    VADLQPQLKI DKAVAFQNPQ THVIENLHAA AYRNALANPL YCPDYRIGKV 200
    TSEELHYFVQ NHFTSARMAL IGLGVSHPVL KQVAEQFLNM RGGLGLSGAK 250
    ANYRGGEIRE QNGDSLVHAA FVAESAVAGS AEANAFSVLQ HVLGAGPHVK 300
    RGSNTTSHLH QAVAKATQQP FDVSAFNASY SDSGLFGIYT ISQATAAGDV 350
    IKAAYNQVKT IAQGNLSNTD VQAAKNKLKA GYLMSVESSE CFLEEVGSQA 400
    LVAGSYMPPS TVLQQIDSVA NADIINAAKK FVSGQKSMAA SGNLGHTPFV 450
    DEL 453
    Length:453
    Mass (Da):48,443
    Last modified:July 11, 2002 - v3
    Checksum:iBFA655C24C07AF52
    GO

    Experimental Info

    Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
    Sequence conflicti360 – 3601T → R in AAA35710. (PubMed:2547763)Curated

    Natural variant

    Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
    Natural varianti148 – 1481R → S.
    Corresponds to variant rs2228473 [ dbSNP | Ensembl ].
    VAR_029336
    Natural varianti183 – 1831R → Q.1 Publication
    Corresponds to variant rs4850 [ dbSNP | Ensembl ].
    VAR_034582
    Natural varianti183 – 1831R → W in MC3DN5. 1 Publication
    VAR_069709
    Natural varianti208 – 2081F → Y in a colorectal cancer sample; somatic mutation. 1 Publication
    VAR_036479
    Natural varianti254 – 2541R → H.
    Corresponds to variant rs11863893 [ dbSNP | Ensembl ].
    VAR_034583

    Sequence databases

    Select the link destinations:
    EMBL
    GenBank
    DDBJ
    Links Updated
    J04973 mRNA. Translation: AAA35710.1.
    AK094006 mRNA. Translation: BAG52796.1.
    CH471249 Genomic DNA. Translation: EAW50592.1.
    BC003136 mRNA. Translation: AAH03136.1.
    BC000484 mRNA. Translation: AAH00484.1.
    CCDSiCCDS10601.1.
    PIRiA32629.
    RefSeqiNP_003357.2. NM_003366.2.
    UniGeneiHs.528803.

    Genome annotation databases

    EnsembliENST00000268379; ENSP00000268379; ENSG00000140740.
    GeneIDi7385.
    KEGGihsa:7385.
    UCSCiuc002djx.3. human.

    Polymorphism databases

    DMDMi21903482.

    Keywords - Coding sequence diversityi

    Polymorphism

    Cross-referencesi

    Sequence databases

    Select the link destinations:
    EMBL
    GenBank
    DDBJ
    Links Updated
    J04973 mRNA. Translation: AAA35710.1 .
    AK094006 mRNA. Translation: BAG52796.1 .
    CH471249 Genomic DNA. Translation: EAW50592.1 .
    BC003136 mRNA. Translation: AAH03136.1 .
    BC000484 mRNA. Translation: AAH00484.1 .
    CCDSi CCDS10601.1.
    PIRi A32629.
    RefSeqi NP_003357.2. NM_003366.2.
    UniGenei Hs.528803.

    3D structure databases

    ProteinModelPortali P22695.
    SMRi P22695. Positions 32-453.
    ModBasei Search...
    MobiDBi Search...

    Protein-protein interaction databases

    BioGridi 113231. 29 interactions.
    IntActi P22695. 9 interactions.
    MINTi MINT-2801885.
    STRINGi 9606.ENSP00000268379.

    Protein family/group databases

    MEROPSi M16.974.

    PTM databases

    PhosphoSitei P22695.

    Polymorphism databases

    DMDMi 21903482.

    2D gel databases

    REPRODUCTION-2DPAGE IPI00305383.
    UCD-2DPAGE P22695.

    Proteomic databases

    MaxQBi P22695.
    PaxDbi P22695.
    PeptideAtlasi P22695.
    PRIDEi P22695.

    Protocols and materials databases

    DNASUi 7385.
    Structural Biology Knowledgebase Search...

    Genome annotation databases

    Ensembli ENST00000268379 ; ENSP00000268379 ; ENSG00000140740 .
    GeneIDi 7385.
    KEGGi hsa:7385.
    UCSCi uc002djx.3. human.

    Organism-specific databases

    CTDi 7385.
    GeneCardsi GC16P021963.
    HGNCi HGNC:12586. UQCRC2.
    HPAi HPA007998.
    HPA019146.
    MIMi 191329. gene.
    615160. phenotype.
    neXtProti NX_P22695.
    Orphaneti 1460. Isolated CoQ-cytochrome C reductase deficiency.
    PharmGKBi PA37217.
    GenAtlasi Search...

    Phylogenomic databases

    eggNOGi COG0612.
    HOGENOMi HOG000046923.
    HOVERGENi HBG055236.
    InParanoidi P22695.
    KOi K00415.
    OMAi QFVQNNF.
    OrthoDBi EOG79CXZF.
    PhylomeDBi P22695.
    TreeFami TF105033.

    Enzyme and pathway databases

    Reactomei REACT_22393. Respiratory electron transport.
    SignaLinki P22695.

    Miscellaneous databases

    ChiTaRSi UQCRC2. human.
    GeneWikii UQCRC2.
    GenomeRNAii 7385.
    NextBioi 28916.
    PROi P22695.
    SOURCEi Search...

    Gene expression databases

    ArrayExpressi P22695.
    Bgeei P22695.
    CleanExi HS_UQCRC2.
    Genevestigatori P22695.

    Family and domain databases

    Gene3Di 3.30.830.10. 2 hits.
    InterProi IPR011249. Metalloenz_LuxS/M16.
    IPR011237. Pept_M16_dom.
    IPR011765. Pept_M16_N.
    IPR001431. Pept_M16_Zn_BS.
    IPR007863. Peptidase_M16_C.
    [Graphical view ]
    Pfami PF00675. Peptidase_M16. 1 hit.
    PF05193. Peptidase_M16_C. 1 hit.
    [Graphical view ]
    SUPFAMi SSF63411. SSF63411. 3 hits.
    PROSITEi PS00143. INSULINASE. 1 hit.
    [Graphical view ]
    ProtoNeti Search...

    Publicationsi

    1. "Complementary DNA encoding core protein II of human mitochondrial cytochrome bc1 complex. Substantial diversity in deduced primary structure from its yeast counterpart."
      Hosokawa Y., Suzuki H., Toda H., Nishikimi M., Ozawa T.
      J. Biol. Chem. 264:13483-13488(1989) [PubMed] [Europe PMC] [Abstract]
      Cited for: NUCLEOTIDE SEQUENCE [MRNA], VARIANT GLN-183.
    2. "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: Uterus.
    3. Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE GENOMIC DNA].
    4. "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: Lung and Skin.
    5. Lubec G., Vishwanath V.
      Submitted (MAR-2007) to UniProtKB
      Cited for: PROTEIN SEQUENCE OF 43-60; 71-84 AND 200-217, IDENTIFICATION BY MASS SPECTROMETRY.
      Tissue: Brain and Cajal-Retzius cell.
    6. Cited for: IDENTIFICATION BY MASS SPECTROMETRY [LARGE SCALE ANALYSIS].
    7. Cited for: VARIANT [LARGE SCALE ANALYSIS] TYR-208.
    8. "Mitochondrial complex III deficiency caused by a homozygous UQCRC2 mutation presenting with neonatal-onset recurrent metabolic decompensation."
      Miyake N., Yano S., Sakai C., Hatakeyama H., Matsushima Y., Shiina M., Watanabe Y., Bartley J., Abdenur J.E., Wang R.Y., Chang R., Tsurusaki Y., Doi H., Nakashima M., Saitsu H., Ogata K., Goto Y., Matsumoto N.
      Hum. Mutat. 34:446-452(2013) [PubMed] [Europe PMC] [Abstract]
      Cited for: VARIANT MC3DN5 TRP-183.

    Entry informationi

    Entry nameiQCR2_HUMAN
    AccessioniPrimary (citable) accession number: P22695
    Secondary accession number(s): B3KSN4, Q9BQ05
    Entry historyi
    Integrated into UniProtKB/Swiss-Prot: August 1, 1991
    Last sequence update: July 11, 2002
    Last modified: October 1, 2014
    This is version 148 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 16
      Human chromosome 16: 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