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P08574

- CY1_HUMAN

UniProt

P08574 - CY1_HUMAN

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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

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
Complete GO annotation...

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
Complete GO annotation...

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. 32 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.
GeneTreeiENSGT00390000012445.
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

« Hide

        10         20         30         40         50
MAAAAASLRG VVLGPRGAGL PGARARGLLC SARPGQLPLR TPQAVALSSK
60 70 80 90 100
SGLSRGRKVM LSALGMLAAG GAGLAMALHS AVSASDLELH PPSYPWSHRG
110 120 130 140 150
LLSSLDHTSI RRGFQVYKQV CASCHSMDFV AYRHLVGVCY TEDEAKELAA
160 170 180 190 200
EVEVQDGPNE DGEMFMRPGK LFDYFPKPYP NSEAARAANN GALPPDLSYI
210 220 230 240 250
VRARHGGEDY VFSLLTGYCE PPTGVSLREG LYFNPYFPGQ AIAMAPPIYT
260 270 280 290 300
DVLEFDDGTP ATMSQIAKDV CTFLRWASEP EHDHRKRMGL KMLMMMALLV
310 320
PLVYTIKRHK WSVLKSRKLA YRPPK
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. 32 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.
GeneTreei ENSGT00390000012445.
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

« Hide 'large scale' publications
  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 29, 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

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