P08574 (CY1_HUMAN) Reviewed, UniProtKB/Swiss-Prot

Names and origin

Protein names

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 names

Name:

CYC1

Organism

Homo sapiens (Human) [Reference proteome]

Taxonomic identifier

9606 [NCBI]

Taxonomic lineage

cellular organisms › Eukaryota › Opisthokonta › Metazoa › Eumetazoa › Bilateria › Deuterostomia › Chordata › Craniata › Vertebrata › Gnathostomata › Teleostomi › Euteleostomi › Sarcopterygii › Tetrapoda › Amniota › Mammalia › Theria › Eutheria › Euarchontoglires › Primates › Haplorrhini › Simiiformes › Catarrhini › Hominoidea › Hominidae › Homininae › Homo

Protein attributes

Sequence length	325 AA.
Sequence status	Complete.
Sequence processing	The displayed sequence is further processed into a mature form.
Protein existence	Evidence at protein level

General annotation (Comments)

Function	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.
Subunit structure	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).
Subcellular location	Mitochondrion inner membrane; Single-pass membrane protein; Intermembrane side.
Post-translational modification	Binds 1 heme group per subunit.
Sequence similarities	Belongs to the cytochrome c family. Contains 1 cytochrome c domain.

Ontologies

Keywords
Biological process	Electron transport Respiratory chain Transport
Cellular component	Membrane Mitochondrion Mitochondrion inner membrane
Coding sequence diversity	Polymorphism
Domain	Transit peptide Transmembrane Transmembrane helix
Ligand	Heme Iron Metal-binding
Technical term	Complete proteome Reference proteome
Gene Ontology (GO)
Biological_process	respiratory electron transport chain Traceable author statement. Source: Reactome small molecule metabolic process Traceable author statement. Source: Reactome
Cellular_component	cell junction Inferred from direct assay. Source: HPA integral to membrane Inferred from electronic annotation. Source: UniProtKB-KW mitochondrial inner membrane Traceable author statement. Source: Reactome respiratory chain Inferred from electronic annotation. Source: UniProtKB-KW
Molecular_function	electron transporter, transferring electrons from CoQH2-cytochrome c reductase complex and cytochrome c oxidase complex activity Traceable author statement Ref.2. Source: ProtInc heme binding Inferred from electronic annotation. Source: InterPro iron ion binding Inferred from electronic annotation. Source: InterPro
Complete GO annotation...

Sequence annotation (Features)

	Feature key	Position(s)	Length	Description	Graphical view	Feature identifier
Molecule processing
	Transit peptide	1 – 84	84	Mitochondrion
	Chain	85 – 325	241	Cytochrome c1, heme protein, mitochondrial		PRO_0000006554
Regions
	Transmembrane	292 – 306	15	Helical; Note=Anchors to the membrane; Potential
	Domain	108 – 209	102	Cytochrome c
Sites
	Metal binding	125	1	Iron (heme axial ligand)
	Metal binding	244	1	Iron (heme axial ligand) By similarity
	Binding site	121	1	Heme (covalent)
	Binding site	124	1	Heme (covalent)
Natural variations
	Natural variant	76	1	M → V. Ref.1 Ref.2 Ref.3 Ref.5 Ref.7 Corresponds to variant rs7820984 [ dbSNP | Ensembl ].		VAR_025163
	Natural variant	89	1	L → V. Ref.1 Ref.10		VAR_013631

Sequences

	Sequence		Length	Mass (Da)
	P08574 [UniParc]. Last modified November 2, 2010. Version 3. Checksum: CC8EA2E60E96BBDC	FASTA	325	35,422
	10 20 30 40 50 60 MAAAAASLRG VVLGPRGAGL PGARARGLLC SARPGQLPLR TPQAVALSSK SGLSRGRKVM 70 80 90 100 110 120 LSALGMLAAG GAGLAMALHS AVSASDLELH PPSYPWSHRG LLSSLDHTSI RRGFQVYKQV 130 140 150 160 170 180 CASCHSMDFV AYRHLVGVCY TEDEAKELAA EVEVQDGPNE DGEMFMRPGK LFDYFPKPYP 190 200 210 220 230 240 NSEAARAANN GALPPDLSYI VRARHGGEDY VFSLLTGYCE PPTGVSLREG LYFNPYFPGQ 250 260 270 280 290 300 AIAMAPPIYT DVLEFDDGTP ATMSQIAKDV CTFLRWASEP EHDHRKRMGL KMLMMMALLV 310 320 PLVYTIKRHK WSVLKSRKLA YRPPK

References

	« Hide 'large scale' referencesShow 'large scale' references »
[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]	"Initial characterization of the human central proteome." Burkard T.R., Planyavsky M., Kaupe I., Breitwieser F.P., Buerckstuemmer T., Bennett K.L., Superti-Furga G., Colinge J. BMC Syst. Biol. 5:17-17(2011) [PubMed] [Europe PMC] [Abstract] 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.
+	Additional computationally mapped references.

Web resources

NIEHS-SNPs

Cross-references

Sequence databases
EMBL GenBank DDBJ	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.
IPI	IPI00029264.
PIR	S00680. A31481.
RefSeq	NP_001907.2. NM_001916.3.
UniGene	Hs.289271.
3D structure databases
ProteinModelPortal	P08574.
SMR	P08574. Positions 85-325.
ModBase	Search...
Protein-protein interaction databases
IntAct	P08574. 5 interactions.
STRING	9606.ENSP00000317159.
PTM databases
PhosphoSite	P08574.
Polymorphism databases
DMDM	311033458.
Proteomic databases
PaxDb	P08574.
PRIDE	P08574.
Protocols and materials databases
DNASU	1537.
StructuralBiologyKnowledgebase	Search...
Genome annotation databases
Ensembl	ENST00000318911; ENSP00000317159; ENSG00000179091.
GeneID	1537.
KEGG	hsa:1537.
UCSC	uc003zay.3. human.
Organism-specific databases
CTD	1537.
GeneCards	GC08P145149.
HGNC	HGNC:2579. CYC1.
HPA	HPA001247.
MIM	123980. gene.
neXtProt	NX_P08574.
PharmGKB	PA27077.
GenAtlas	Search...
Phylogenomic databases
eggNOG	COG2857.
HOGENOM	HOG000003867.
HOVERGEN	HBG001239.
InParanoid	P08574.
KO	K00413.
OMA	MAPPIYT.
OrthoDB	EOG4001JZ.
PhylomeDB	P08574.
Enzyme and pathway databases
Reactome	REACT_111217. Metabolism. REACT_17015. Metabolism of proteins.
Gene expression databases
Bgee	P08574.
CleanEx	HS_CYC1.
Genevestigator	P08574.
GermOnline	ENSG00000179091. Homo sapiens.
Family and domain databases
InterPro	IPR002326. Cyt_c1. IPR021157. Cyt_c1_TM_anchor_C. IPR009056. Cyt_c_dom. [Graphical view]
PANTHER	PTHR10266. PTHR10266. 1 hit.
Pfam	PF02167. Cytochrom_C1. 1 hit. [Graphical view]
PRINTS	PR00603. CYTOCHROMEC1.
SUPFAM	SSF81496. Cyt_c1_TM_anchor_C. 1 hit. SSF46626. Cytochrome_c. 1 hit.
PROSITE	PS51007. CYTC. False negative. [Graphical view]
ProtoNet	Search...
Other
ChiTaRS	CYC1. human.
GenomeRNAi	1537.
NextBio	6357.
SOURCE	Search...

Entry information

Entry name

CY1_HUMAN

Accession

Primary (citable) accession number: P08574
Secondary accession number(s): Q5U062, Q6FHS7

Entry history

Integrated into UniProtKB/Swiss-Prot:	August 1, 1988
Last sequence update:	November 2, 2010
Last modified:	April 3, 2013
This is version 133 of the entry and version 3 of the sequence. [Complete history]

Entry status

Reviewed (UniProtKB/Swiss-Prot)

Annotation program

Chordata Protein Annotation Program

Disclaimer

Any 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.