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P08574

- CY1_HUMAN

UniProt

P08574 - CY1_HUMAN

Protein

Cytochrome c1, heme protein, mitochondrial

Gene

CYC1

Organism
Homo sapiens (Human)
Status
Reviewed - Annotation score: 5 out of 5- Experimental evidence at protein leveli
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    • History
      Entry version 147 (01 Oct 2014)
      Sequence version 3 (02 Nov 2010)
      Previous versions | rss
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    Functioni

    This is the heme-containing component of the cytochrome b-c1 complex, which accepts electrons from Rieske protein and transfers electrons to cytochrome c in the mitochondrial respiratory chain.

    Sites

    Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
    Binding sitei121 – 1211Heme (covalent)
    Binding sitei124 – 1241Heme (covalent)
    Metal bindingi125 – 1251Iron (heme axial ligand)
    Metal bindingi244 – 2441Iron (heme axial ligand)By similarity

    GO - Molecular functioni

    1. electron transporter, transferring electrons from CoQH2-cytochrome c reductase complex and cytochrome c oxidase complex activity Source: ProtInc
    2. heme binding Source: InterPro
    3. iron ion binding Source: InterPro

    GO - Biological processi

    1. cellular metabolic process Source: Reactome
    2. respiratory electron transport chain Source: Reactome
    3. response to glucagon Source: Ensembl
    4. small molecule metabolic process Source: Reactome

    Keywords - Biological processi

    Electron transport, Respiratory chain, Transport

    Keywords - Ligandi

    Heme, Iron, Metal-binding

    Enzyme and pathway databases

    ReactomeiREACT_118595. Mitochondrial protein import.
    REACT_22393. Respiratory electron transport.

    Names & Taxonomyi

    Protein namesi
    Recommended name:
    Cytochrome c1, heme protein, mitochondrial
    Alternative name(s):
    Complex III subunit 4
    Complex III subunit IV
    Cytochrome b-c1 complex subunit 4
    Ubiquinol-cytochrome-c reductase complex cytochrome c1 subunit
    Short name:
    Cytochrome c-1
    Gene namesi
    Name:CYC1
    OrganismiHomo sapiens (Human)
    Taxonomic identifieri9606 [NCBI]
    Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresPrimatesHaplorrhiniCatarrhiniHominidaeHomo
    ProteomesiUP000005640: Chromosome 8

    Organism-specific databases

    HGNCiHGNC:2579. CYC1.

    Subcellular locationi

    GO - Cellular componenti

    1. integral component of membrane Source: UniProtKB-KW
    2. membrane Source: UniProtKB
    3. mitochondrial inner membrane Source: Reactome
    4. mitochondrion Source: UniProt
    5. nucleus Source: UniProt
    6. respiratory chain Source: UniProtKB-KW

    Keywords - Cellular componenti

    Membrane, Mitochondrion, Mitochondrion inner membrane

    Pathology & Biotechi

    Involvement in diseasei

    Mitochondrial complex III deficiency, nuclear 6 (MC3DN6) [MIM:615453]: An autosomal recessive disorder caused by mitochondrial dysfunction. It is characterized by onset in early childhood of episodic acute lactic acidosis, ketoacidosis, and insulin-responsive hyperglycemia, usually associated with infection. Laboratory studies show decreased activity of mitochondrial complex III. Psychomotor development is normal.1 Publication
    Note: The disease is caused by mutations affecting the gene represented in this entry.
    Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
    Natural varianti96 – 961W → C in MC3DN6. 1 Publication
    VAR_070847
    Natural varianti215 – 2151L → F in MC3DN6. 1 Publication
    VAR_070848

    Keywords - Diseasei

    Disease mutation

    Organism-specific databases

    MIMi615453. phenotype.
    Orphaneti1460. Isolated CoQ-cytochrome C reductase deficiency.
    PharmGKBiPA27077.

    PTM / Processingi

    Molecule processing

    Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
    Transit peptidei1 – 8484MitochondrionAdd
    BLAST
    Chaini85 – 325241Cytochrome c1, heme protein, mitochondrialPRO_0000006554Add
    BLAST

    Post-translational modificationi

    Binds 1 heme group per subunit.

    Proteomic databases

    MaxQBiP08574.
    PaxDbiP08574.
    PRIDEiP08574.

    PTM databases

    PhosphoSiteiP08574.

    Expressioni

    Gene expression databases

    BgeeiP08574.
    CleanExiHS_CYC1.
    GenevestigatoriP08574.

    Organism-specific databases

    HPAiHPA001247.

    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

    BioGridi107917. 27 interactions.
    IntActiP08574. 8 interactions.
    MINTiMINT-3006365.
    STRINGi9606.ENSP00000317159.

    Structurei

    3D structure databases

    ProteinModelPortaliP08574.
    SMRiP08574. Positions 85-325.
    ModBaseiSearch...
    MobiDBiSearch...

    Transmembrane

    Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
    Transmembranei292 – 30615Helical; Note=Anchors to the membraneSequence AnalysisAdd
    BLAST

    Family & Domainsi

    Domains and Repeats

    Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
    Domaini108 – 209102Cytochrome cAdd
    BLAST

    Sequence similaritiesi

    Belongs to the cytochrome c family.Curated
    Contains 1 cytochrome c domain.Curated

    Keywords - Domaini

    Transit peptide, Transmembrane, Transmembrane helix

    Phylogenomic databases

    eggNOGiCOG2857.
    HOGENOMiHOG000003867.
    HOVERGENiHBG001239.
    InParanoidiP08574.
    KOiK00413.
    OMAiGLHPPAY.
    OrthoDBiEOG7GBFX7.
    PhylomeDBiP08574.
    TreeFamiTF314799.

    Family and domain databases

    Gene3Di1.10.760.10. 1 hit.
    InterProiIPR009056. Cyt_c-like_dom.
    IPR002326. Cyt_c1.
    IPR021157. Cyt_c1_TM_anchor_C.
    [Graphical view]
    PANTHERiPTHR10266. PTHR10266. 1 hit.
    PfamiPF02167. Cytochrom_C1. 1 hit.
    [Graphical view]
    PRINTSiPR00603. CYTOCHROMEC1.
    SUPFAMiSSF46626. SSF46626. 1 hit.
    SSF81496. SSF81496. 1 hit.

    Sequencei

    Sequence statusi: Complete.

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

    P08574-1 [UniParc]FASTAAdd to Basket

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    MAAAAASLRG VVLGPRGAGL PGARARGLLC SARPGQLPLR TPQAVALSSK    50
    SGLSRGRKVM LSALGMLAAG GAGLAMALHS AVSASDLELH PPSYPWSHRG 100
    LLSSLDHTSI RRGFQVYKQV CASCHSMDFV AYRHLVGVCY TEDEAKELAA 150
    EVEVQDGPNE DGEMFMRPGK LFDYFPKPYP NSEAARAANN GALPPDLSYI 200
    VRARHGGEDY VFSLLTGYCE PPTGVSLREG LYFNPYFPGQ AIAMAPPIYT 250
    DVLEFDDGTP ATMSQIAKDV CTFLRWASEP EHDHRKRMGL KMLMMMALLV 300
    PLVYTIKRHK WSVLKSRKLA YRPPK 325
    Length:325
    Mass (Da):35,422
    Last modified:November 2, 2010 - v3
    Checksum:iCC8EA2E60E96BBDC
    GO

    Natural variant

    Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
    Natural varianti76 – 761M → V.5 Publications
    Corresponds to variant rs7820984 [ dbSNP | Ensembl ].
    VAR_025163
    Natural varianti89 – 891L → V.2 Publications
    VAR_013631
    Natural varianti96 – 961W → C in MC3DN6. 1 Publication
    VAR_070847
    Natural varianti215 – 2151L → F in MC3DN6. 1 Publication
    VAR_070848

    Sequence databases

    Select the link destinations:
    EMBL
    GenBank
    DDBJ
    Links Updated
    M16597 mRNA. Translation: AAA35730.1.
    J04444 Genomic DNA. Translation: AAA52135.1.
    CR541674 mRNA. Translation: CAG46475.1.
    BT019798 mRNA. Translation: AAV38601.1.
    DQ300360 Genomic DNA. Translation: ABB96244.1.
    AC104592 Genomic DNA. No translation available.
    BC001006 mRNA. Translation: AAH01006.1.
    BC015616 mRNA. Translation: AAH15616.1.
    BC020566 mRNA. Translation: AAH20566.1.
    X06994 mRNA. Translation: CAA30052.1.
    CCDSiCCDS6415.1.
    PIRiA31481. S00680.
    RefSeqiNP_001907.2. NM_001916.4.
    UniGeneiHs.289271.

    Genome annotation databases

    EnsembliENST00000318911; ENSP00000317159; ENSG00000179091.
    GeneIDi1537.
    KEGGihsa:1537.
    UCSCiuc003zay.3. human.

    Polymorphism databases

    DMDMi311033458.

    Keywords - Coding sequence diversityi

    Polymorphism

    Cross-referencesi

    Web resourcesi

    NIEHS-SNPs

    Sequence databases

    Select the link destinations:
    EMBL
    GenBank
    DDBJ
    Links Updated
    M16597 mRNA. Translation: AAA35730.1 .
    J04444 Genomic DNA. Translation: AAA52135.1 .
    CR541674 mRNA. Translation: CAG46475.1 .
    BT019798 mRNA. Translation: AAV38601.1 .
    DQ300360 Genomic DNA. Translation: ABB96244.1 .
    AC104592 Genomic DNA. No translation available.
    BC001006 mRNA. Translation: AAH01006.1 .
    BC015616 mRNA. Translation: AAH15616.1 .
    BC020566 mRNA. Translation: AAH20566.1 .
    X06994 mRNA. Translation: CAA30052.1 .
    CCDSi CCDS6415.1.
    PIRi A31481. S00680.
    RefSeqi NP_001907.2. NM_001916.4.
    UniGenei Hs.289271.

    3D structure databases

    ProteinModelPortali P08574.
    SMRi P08574. Positions 85-325.
    ModBasei Search...
    MobiDBi Search...

    Protein-protein interaction databases

    BioGridi 107917. 27 interactions.
    IntActi P08574. 8 interactions.
    MINTi MINT-3006365.
    STRINGi 9606.ENSP00000317159.

    PTM databases

    PhosphoSitei P08574.

    Polymorphism databases

    DMDMi 311033458.

    Proteomic databases

    MaxQBi P08574.
    PaxDbi P08574.
    PRIDEi P08574.

    Protocols and materials databases

    DNASUi 1537.
    Structural Biology Knowledgebase Search...

    Genome annotation databases

    Ensembli ENST00000318911 ; ENSP00000317159 ; ENSG00000179091 .
    GeneIDi 1537.
    KEGGi hsa:1537.
    UCSCi uc003zay.3. human.

    Organism-specific databases

    CTDi 1537.
    GeneCardsi GC08P145149.
    HGNCi HGNC:2579. CYC1.
    HPAi HPA001247.
    MIMi 123980. gene.
    615453. phenotype.
    neXtProti NX_P08574.
    Orphaneti 1460. Isolated CoQ-cytochrome C reductase deficiency.
    PharmGKBi PA27077.
    GenAtlasi Search...

    Phylogenomic databases

    eggNOGi COG2857.
    HOGENOMi HOG000003867.
    HOVERGENi HBG001239.
    InParanoidi P08574.
    KOi K00413.
    OMAi GLHPPAY.
    OrthoDBi EOG7GBFX7.
    PhylomeDBi P08574.
    TreeFami TF314799.

    Enzyme and pathway databases

    Reactomei REACT_118595. Mitochondrial protein import.
    REACT_22393. Respiratory electron transport.

    Miscellaneous databases

    ChiTaRSi CYC1. human.
    GeneWikii CYC1.
    GenomeRNAii 1537.
    NextBioi 6357.
    PROi P08574.
    SOURCEi Search...

    Gene expression databases

    Bgeei P08574.
    CleanExi HS_CYC1.
    Genevestigatori P08574.

    Family and domain databases

    Gene3Di 1.10.760.10. 1 hit.
    InterProi IPR009056. Cyt_c-like_dom.
    IPR002326. Cyt_c1.
    IPR021157. Cyt_c1_TM_anchor_C.
    [Graphical view ]
    PANTHERi PTHR10266. PTHR10266. 1 hit.
    Pfami PF02167. Cytochrom_C1. 1 hit.
    [Graphical view ]
    PRINTSi PR00603. CYTOCHROMEC1.
    SUPFAMi SSF46626. SSF46626. 1 hit.
    SSF81496. SSF81496. 1 hit.
    ProtoNeti Search...

    Publicationsi

    1. "Nucleotide sequence of a cDNA encoding the precursor to human cytochrome c1."
      Nishikimi M., Ohta S., Suzuki H., Tanaka T., Kikkawa F., Tanaka M., Kagawa Y., Ozawa T.
      Nucleic Acids Res. 16:3577-3577(1988) [PubMed] [Europe PMC] [Abstract]
      Cited for: NUCLEOTIDE SEQUENCE [MRNA], VARIANTS VAL-76 AND VAL-89.
    2. "Structural organization of the human mitochondrial cytochrome c1 gene."
      Suzuki H., Hosokawa Y., Nishikimi M., Ozawa T.
      J. Biol. Chem. 264:1368-1374(1989) [PubMed] [Europe PMC] [Abstract]
      Cited for: NUCLEOTIDE SEQUENCE [GENOMIC DNA], VARIANT VAL-76.
    3. "Cloning of human full open reading frames in Gateway(TM) system entry vector (pDONR201)."
      Halleck A., Ebert L., Mkoundinya M., Schick M., Eisenstein S., Neubert P., Kstrang K., Schatten R., Shen B., Henze S., Mar W., Korn B., Zuo D., Hu Y., LaBaer J.
      Submitted (JUN-2004) to the EMBL/GenBank/DDBJ databases
      Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE MRNA], VARIANT VAL-76.
    4. "Cloning of human full-length CDSs in BD Creator(TM) system donor vector."
      Kalnine N., Chen X., Rolfs A., Halleck A., Hines L., Eisenstein S., Koundinya M., Raphael J., Moreira D., Kelley T., LaBaer J., Lin Y., Phelan M., Farmer A.
      Submitted (OCT-2004) to the EMBL/GenBank/DDBJ databases
      Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE MRNA].
    5. NIEHS SNPs program
      Submitted (NOV-2005) to the EMBL/GenBank/DDBJ databases
      Cited for: NUCLEOTIDE SEQUENCE [GENOMIC DNA], VARIANT VAL-76.
    6. "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].
    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], VARIANT VAL-76.
      Tissue: Brain, Lung and Skin.
    8. "Isolation of a cDNA clone for human cytochrome c1 from a lambda gt11 expression library."
      Nishikimi M., Suzuki H., Ohta S., Sakurai T., Shimomura Y., Tanaka M., Kagawa Y., Ozawa T.
      Biochem. Biophys. Res. Commun. 145:34-39(1987) [PubMed] [Europe PMC] [Abstract]
      Cited for: NUCLEOTIDE SEQUENCE [MRNA] OF 99-325.
    9. Cited for: IDENTIFICATION BY MASS SPECTROMETRY [LARGE SCALE ANALYSIS].
    10. "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 VAL-89.
    11. Cited for: VARIANTS MC3DN6 CYS-96 AND PHE-215.

    Entry informationi

    Entry nameiCY1_HUMAN
    AccessioniPrimary (citable) accession number: P08574
    Secondary accession number(s): Q5U062, Q6FHS7
    Entry historyi
    Integrated into UniProtKB/Swiss-Prot: August 1, 1988
    Last sequence update: November 2, 2010
    Last modified: October 1, 2014
    This is version 147 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

    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