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Protein

Cytochrome c1, heme protein, mitochondrial

Gene

CYC1

Organism
Homo sapiens (Human)
Status
Reviewed-Annotation score: -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)DescriptionActionsGraphical viewLength
Binding sitei121Heme (covalent)1
Binding sitei124Heme (covalent)1
Metal bindingi125Iron (heme axial ligand)1
Metal bindingi244Iron (heme axial ligand)By similarity1

GO - Molecular functioni

GO - Biological processi

Keywordsi

Biological processElectron transport, Respiratory chain, Transport
LigandHeme, Iron, Metal-binding

Enzyme and pathway databases

ReactomeiR-HSA-1268020 Mitochondrial protein import
R-HSA-611105 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
Proteomesi
  • UP000005640 Componenti: Chromosome 8

Organism-specific databases

EuPathDBiHostDB:ENSG00000179091.4
HGNCiHGNC:2579 CYC1
MIMi123980 gene
neXtProtiNX_P08574

Subcellular locationi

Extracellular region or secreted Cytosol Plasma membrane Cytoskeleton Lysosome Endosome Peroxisome ER Golgi apparatus Nucleus Mitochondrion Manual annotation Automatic computational assertionGraphics by Christian Stolte; Source: COMPARTMENTS

Topology

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Transmembranei292 – 306Helical; Note=Anchors to the membraneSequence analysisAdd BLAST15

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)DescriptionActionsGraphical viewLength
Natural variantiVAR_07084796W → C in MC3DN6. 1 PublicationCorresponds to variant dbSNP:rs587777041Ensembl.1
Natural variantiVAR_070848215L → F in MC3DN6. 1 PublicationCorresponds to variant dbSNP:rs587777042Ensembl.1

Keywords - Diseasei

Disease mutation, Primary mitochondrial disease

Organism-specific databases

DisGeNETi1537
MalaCardsiCYC1
MIMi615453 phenotype
OpenTargetsiENSG00000179091
Orphaneti1460 Isolated CoQ-cytochrome C reductase deficiency
PharmGKBiPA27077

Chemistry databases

DrugBankiDB08453 2-NONYL-4-HYDROXYQUINOLINE N-OXIDE
DB07778 FAMOXADONE
DB07401 METHYL (2Z)-2-(2-{[6-(2-CYANOPHENOXY)PYRIMIDIN-4-YL]OXY}PHENYL)-3-METHOXYACRYLATE
DB08330 METHYL (2Z)-3-METHOXY-2-{2-[(E)-2-PHENYLVINYL]PHENYL}ACRYLATE
DB08690 UBIQUINONE-2

Polymorphism and mutation databases

BioMutaiCYC1
DMDMi311033458

PTM / Processingi

Molecule processing

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Transit peptidei1 – 84MitochondrionCombined sourcesAdd BLAST84
ChainiPRO_000000655485 – 325Cytochrome c1, heme protein, mitochondrialAdd BLAST241

Amino acid modifications

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Modified residuei182PhosphoserineCombined sources1

Post-translational modificationi

Binds 1 heme group per subunit.

Keywords - PTMi

Phosphoprotein

Proteomic databases

EPDiP08574
MaxQBiP08574
PaxDbiP08574
PeptideAtlasiP08574
PRIDEiP08574
TopDownProteomicsiP08574

PTM databases

iPTMnetiP08574
PhosphoSitePlusiP08574
SwissPalmiP08574

Expressioni

Gene expression databases

BgeeiENSG00000179091
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

BioGridi10791783 interactors.
IntActiP08574 12 interactors.
MINTiP08574
STRINGi9606.ENSP00000317159

Structurei

Secondary structure

1325
Legend: HelixTurnBeta strandPDB Structure known for this area
Show more details
Feature keyPosition(s)DescriptionActionsGraphical viewLength
Helixi97 – 99Combined sources3
Beta strandi100 – 102Combined sources3
Helixi107 – 117Combined sources11
Turni118 – 123Combined sources6
Beta strandi131 – 134Combined sources4
Helixi135 – 138Combined sources4
Helixi142 – 149Combined sources8
Beta strandi159 – 162Combined sources4
Beta strandi178 – 181Combined sources4
Helixi182 – 187Combined sources6
Turni188 – 191Combined sources4
Helixi200 – 203Combined sources4
Turni205 – 207Combined sources3
Helixi208 – 216Combined sources9
Beta strandi242 – 244Combined sources3
Helixi263 – 278Combined sources16
Helixi282 – 315Combined sources34

3D structure databases

Select the link destinations:
PDBei
RCSB PDBi
PDBji
Links Updated
PDB entryMethodResolution (Å)ChainPositionsPDBsum
5XTEelectron microscopy3.40H/U85-325[»]
5XTHelectron microscopy3.90AH/AU85-325[»]
5XTIelectron microscopy17.40AH/AU85-325[»]
ProteinModelPortaliP08574
SMRiP08574
ModBaseiSearch...
MobiDBiSearch...

Family & Domainsi

Domains and Repeats

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Domaini108 – 209Cytochrome cAdd BLAST102

Sequence similaritiesi

Belongs to the cytochrome c family.Curated

Keywords - Domaini

Transit peptide, Transmembrane, Transmembrane helix

Phylogenomic databases

eggNOGiKOG3052 Eukaryota
COG2857 LUCA
GeneTreeiENSGT00390000012445
HOGENOMiHOG000003867
HOVERGENiHBG001239
InParanoidiP08574
KOiK00413
OMAiSRKIAYR
OrthoDBiEOG091G0FT2
PhylomeDBiP08574
TreeFamiTF314799

Family and domain databases

Gene3Di1.10.760.101 hit
InterProiView protein in InterPro
IPR036909 Cyt_c-like_dom_sf
IPR002326 Cyt_c1
IPR021157 Cyt_c1_TM_anchor_C
PANTHERiPTHR10266 PTHR10266, 1 hit
PfamiView protein in Pfam
PF02167 Cytochrom_C1, 1 hit
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)DescriptionActionsGraphical viewLength
Natural variantiVAR_02516376M → V5 PublicationsCorresponds to variant dbSNP:rs7820984Ensembl.1
Natural variantiVAR_01363189L → V2 Publications1
Natural variantiVAR_07084796W → C in MC3DN6. 1 PublicationCorresponds to variant dbSNP:rs587777041Ensembl.1
Natural variantiVAR_070848215L → F in MC3DN6. 1 PublicationCorresponds to variant dbSNP:rs587777042Ensembl.1

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
UCSCiuc003zaz.6 human

Keywords - Coding sequence diversityi

Polymorphism

Similar proteinsi

Entry informationi

Entry nameiCY1_HUMAN
AccessioniPrimary (citable) accession number: P08574
Secondary accession number(s): Q5U062, Q6FHS7
Entry historyiIntegrated into UniProtKB/Swiss-Prot: August 1, 1988
Last sequence update: November 2, 2010
Last modified: February 28, 2018
This is version 180 of the entry and version 3 of the sequence. See 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

3D-structure, Complete proteome, Reference proteome