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Protein

Cytochrome c1, heme protein, mitochondrial

Gene

CYC1

Organism
Homo sapiens (Human)
Status
Reviewed-Annotation score: 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

GO - Biological processi

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 Componenti: Chromosome 8

Organism-specific databases

HGNCiHGNC:2579. CYC1.

Subcellular locationi

Topology

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

GO - Cellular componenti

  • integral component of membrane Source: UniProtKB-KW
  • membrane Source: UniProtKB
  • mitochondrial inner membrane Source: Reactome
  • mitochondrion Source: UniProtKB
  • nucleus Source: UniProtKB
  • 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)1 Publication

The disease is caused by mutations affecting the gene represented in this entry.

Disease descriptionAn 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.

See also OMIM:615453
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.

Polymorphism and mutation databases

BioMutaiCYC1.
DMDMi311033458.

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.
GenevisibleiP08574. HS.

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. 31 interactions.
IntActiP08574. 8 interactions.
MINTiMINT-3006365.
STRINGi9606.ENSP00000317159.

Structurei

3D structure databases

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

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.
OMAiASCHSMD.
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:
EMBLi
GenBanki
DDBJi
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.

Keywords - Coding sequence diversityi

Polymorphism

Cross-referencesi

Web resourcesi

NIEHS-SNPs

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
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.

3D structure databases

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

Protein-protein interaction databases

BioGridi107917. 31 interactions.
IntActiP08574. 8 interactions.
MINTiMINT-3006365.
STRINGi9606.ENSP00000317159.

PTM databases

PhosphoSiteiP08574.

Polymorphism and mutation databases

BioMutaiCYC1.
DMDMi311033458.

Proteomic databases

MaxQBiP08574.
PaxDbiP08574.
PRIDEiP08574.

Protocols and materials databases

DNASUi1537.
Structural Biology KnowledgebaseSearch...

Genome annotation databases

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

Organism-specific databases

CTDi1537.
GeneCardsiGC08P145149.
HGNCiHGNC:2579. CYC1.
HPAiHPA001247.
MIMi123980. gene.
615453. phenotype.
neXtProtiNX_P08574.
Orphaneti1460. Isolated CoQ-cytochrome C reductase deficiency.
PharmGKBiPA27077.
GenAtlasiSearch...

Phylogenomic databases

eggNOGiCOG2857.
GeneTreeiENSGT00390000012445.
HOGENOMiHOG000003867.
HOVERGENiHBG001239.
InParanoidiP08574.
KOiK00413.
OMAiASCHSMD.
OrthoDBiEOG7GBFX7.
PhylomeDBiP08574.
TreeFamiTF314799.

Enzyme and pathway databases

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

Miscellaneous databases

ChiTaRSiCYC1. human.
GeneWikiiCYC1.
GenomeRNAii1537.
NextBioi6357.
PROiP08574.
SOURCEiSearch...

Gene expression databases

BgeeiP08574.
CleanExiHS_CYC1.
GenevisibleiP08574. HS.

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.
ProtoNetiSearch...

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. "An enzyme assisted RP-RPLC approach for in-depth analysis of human liver phosphoproteome."
    Bian Y., Song C., Cheng K., Dong M., Wang F., Huang J., Sun D., Wang L., Ye M., Zou H.
    J. Proteomics 96:253-262(2014) [PubMed] [Europe PMC] [Abstract]
    Cited for: IDENTIFICATION BY MASS SPECTROMETRY [LARGE SCALE ANALYSIS].
    Tissue: Liver.
  11. "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.
  12. 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: July 22, 2015
This is version 155 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

Similar proteinsi

Links to similar proteins from the UniProt Reference Clusters (UniRef) at 100%, 90% and 50% sequence identity:
100%UniRef100 combines identical sequences and sub-fragments with 11 or more residues from any organism into Uniref entry.
90%UniRef90 is built by clustering UniRef100 sequences that have at least 90% sequence identity to, and 80% overlap with, the longest sequence (a.k.a seed sequence).
50%UniRef50 is built by clustering UniRef90 seed sequences that have at least 50% sequence identity to, and 80% overlap with, the longest sequence in the cluster.