Reviewed, UniProtKB/Swiss-Prot P31930 (QCR1_HUMAN)

Names and origin

Protein names

Recommended name:
    Cytochrome b-c1 complex subunit 1, mitochondrial
Alternative name(s):
    Ubiquinol-cytochrome-c reductase complex core protein 1
      Short name=Core protein I
    Complex III subunit 1

Gene names

Name:

UQCRC1

Organism

Homo sapiens (Human)

Taxonomic identifier

9606 [NCBI]

Taxonomic lineage

Eukaryota › Metazoa › Chordata › Craniata › Vertebrata › Euteleostomi › Mammalia › Eutheria › Euarchontoglires › Primates › Haplorrhini › Catarrhini › Hominidae › Homo

Protein attributes

Sequence length	480 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 a component of the ubiquinol-cytochrome c reductase complex (complex III or cytochrome b-c1 complex), which is part of the mitochondrial respiratory chain. This protein may mediate formation of the complex between cytochromes c and c1.
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.
Sequence similarities	Belongs to the peptidase M16 family. UQCRC1/QCR1 subfamily.
Caution	Does not seem to have a protease activity as it lack the zinc-binding site.

Ontologies

Keywords
Biological process	Electron transport Respiratory chain Transport
Cellular component	Membrane Mitochondrion Mitochondrion inner membrane
Coding sequence diversity	Polymorphism
Domain	Transit peptide
PTM	Acetylation Phosphoprotein
Technical term	Direct protein sequencing
Gene Ontology (GO)
Biological process	aerobic respiration Ref.1 Traceable author statement. Source: ProtInc proteolysis Inferred from electronic annotation. Source: InterPro transport Inferred from electronic annotation. Source: UniProtKB-KW
Cellular component	mitochondrial respiratory chain Ref.1 Traceable author statement. Source: ProtInc
Molecular function	metalloendopeptidase activity Inferred from electronic annotation. Source: InterPro protein binding Inferred from physical interaction. Source: IntAct ubiquinol-cytochrome-c reductase activity Ref.1 Traceable author statement. Source: ProtInc zinc ion binding Inferred from electronic annotation. Source: InterPro
Complete GO annotation...

Binary interactions

With	Entry	#Exp.	IntAct	Notes
DNAJA1	P31689	1	EBI-1052596,EBI-347834
RUVBL2	Q9Y230	1	EBI-1052596,EBI-352939
SNRPF	P62306	1	EBI-1052596,EBI-356900
SRPRB	Q9Y5M8	1	EBI-1052596,EBI-355393
UQCRC2	P22695	1	EBI-1052596,EBI-1051424

Sequence annotation (Features)

	Feature key	Position(s)	Length	Description	Graphical view	Feature identifier
Molecule processing
	Transit peptide	1 – 34	34	Mitochondrion Ref.6
	Chain	35 – 480	446	Cytochrome b-c1 complex subunit 1, mitochondrial		PRO_0000026786
Amino acid modifications
	Modified residue	138	1	N6-acetyllysine By similarity
	Modified residue	248	1	N6-acetyllysine By similarity
	Modified residue	381	1	Phosphothreonine Ref.9
Natural variations
	Natural variant	215	1	D → H: dbSNP rs17080284.		VAR_034581
	Natural variant	301	1	N → S Ref.1 Ref.11		VAR_013629

Sequences

	Sequence		Length	Mass (Da)
	P31930-1 [UniParc]. Last modified April 4, 2006. Version 3. Checksum: E76B082166CAF48F	FASTA	480	52,646
	10 20 30 40 50 60 MAASVVCRAA TAGAQVLLRA RRSPALLRTP ALRSTATFAQ ALQFVPETQV SLLDNGLRVA 70 80 90 100 110 120 SEQSSQPTCT VGVWIDVGSR FETEKNNGAG YFLEHLAFKG TKNRPGSALE KEVESMGAHL 130 140 150 160 170 180 NAYSTREHTA YYIKALSKDL PKAVELLGDI VQNCSLEDSQ IEKERDVILR EMQENDASMR 190 200 210 220 230 240 DVVFNYLHAT AFQGTPLAQA VEGPSENVRK LSRADLTEYL STHYKAPRMV LAAAGGVEHQ 250 260 270 280 290 300 QLLDLAQKHL GGIPWTYAED AVPTLTPCRF TGSEIRHRDD ALPFAHVAIA VEGPGWASPD 310 320 330 340 350 360 NVALQVANAI IGHYDCTYGG GVHLSSPLAS GAVANKLCQS FQTFSICYAE TGLLGAHFVC 370 380 390 400 410 420 DRMKIDDMMF VLQGQWMRLC TSATESEVAR GKNILRNALV SHLDGTTPVC EDIGRSLLTY 430 440 450 460 470 480 GRRIPLAEWE SRIAEVDASV VREICSKYIY DQCPAVAGYG PIEQLPDYNR IRSGMFWLRF

References

	« Hide 'large scale' referencesShow 'large scale' references »
[1]	"Complete coding sequence, intron/exon organization, and chromosomal location of the gene for the core I protein of human ubiquinol-cytochrome c reductase." Hoffman G.G., Lee S., Christiano A.M., Chung-Honet L.C., Cheng W., Katchman S., Uitto J., Greenspan D.S. J. Biol. Chem. 268:21113-21119(1993) [PubMed: 8407948] [Abstract] Cited for: NUCLEOTIDE SEQUENCE [MRNA], VARIANT SER-301. Tissue: Placenta.
[2]	"A complete cDNA sequence for core I protein subunit of human ubiquinol-cytochrome c reductase." Islam M.M., Tanaka M., Suzuki H., Torii K., Hattori N., Ozawa T. Biochem. Mol. Biol. Int. 32:797-805(1994) [PubMed: 8069229] [Abstract] Cited for: NUCLEOTIDE SEQUENCE [MRNA]. Tissue: Fibroblast.
[3]	Erratum Islam M.M., Tanaka M., Suzuki H., Torii K., Hattori N., Ozawa T. Biochem. Mol. Biol. Int. 33:410-410(1994) [PubMed: 7951059] [Abstract]
[4]	Erratum Islam M.M., Tanaka M., Suzuki H., Torii K., Hattori N., Ozawa T. Biochem. Mol. Biol. Int. 33:815-815(1994) [PubMed: 7981668] [Abstract]
[5]	"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: 15489334] [Abstract] Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE MRNA]. Tissue: Bone marrow.
[6]	"Human liver protein map: update 1993." Hughes G.J., Frutiger S., Paquet N., Pasquali C., Sanchez J.-C., Tissot J.-D., Bairoch A., Appel R.D., Hochstrasser D.F. Electrophoresis 14:1216-1222(1993) [PubMed: 8313870] [Abstract] Cited for: PROTEIN SEQUENCE OF 35-45. Tissue: Liver.
[7]	Lubec G., Vishwanath V., Chen W.-Q., Sun Y. Submitted (DEC-2008) to UniProtKB Cited for: PROTEIN SEQUENCE OF 86-99; 214-225; 229-248; 397-415; 424-442; 448-470 AND 473-479, MASS SPECTROMETRY. Tissue: Brain, Cajal-Retzius cell and Fetal brain cortex.
[8]	"Vectorial proteomics reveal targeting, phosphorylation and specific fragmentation of polymerase I and transcript release factor (PTRF) at the surface of caveolae in human adipocytes." Aboulaich N., Vainonen J.P., Stralfors P., Vener A.V. Biochem. J. 383:237-248(2004) [PubMed: 15242332] [Abstract] Cited for: PROTEIN SEQUENCE OF 143-165; 181-209 AND 397-415. Tissue: Adipocyte.
[9]	"Improved titanium dioxide enrichment of phosphopeptides from HeLa cells and high confident phosphopeptide identification by cross-validation of MS/MS and MS/MS/MS spectra." Yu L.-R., Zhu Z., Chan K.C., Issaq H.J., Dimitrov D.S., Veenstra T.D. J. Proteome Res. 6:4150-4162(2007) [PubMed: 17924679] [Abstract] Cited for: PHOSPHORYLATION [LARGE SCALE ANALYSIS] AT THR-381, MASS SPECTROMETRY. Tissue: Epithelium.
[10]	Colinge J., Superti-Furga G., Bennett K.L. Submitted (OCT-2008) to UniProtKB Cited for: IDENTIFICATION [LARGE SCALE ANALYSIS], MASS SPECTROMETRY.
[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: 10453733] [Abstract] Cited for: VARIANT SER-301.
+	Additional computationally mapped references.

Cross-references

Sequence databases
EMBLGenBankDDBJ	L16842 mRNA. Translation: AAA20046.1. D26485 mRNA. Translation: BAA05495.1. BC009586 mRNA. Translation: AAH09586.1.
IPI	IPI00013847.
PIR	A48043.
RefSeq	NP_003356.2.
UniGene	Hs.119251 Hs.476218
3D structure databases
HSSP	HSSP built from PDB template 1L0N based on UniProtKB P31800.
SMR	P31930. Positions 35-476.
ModBase	Search...
Protein-protein interaction databases
IntAct	P31930. 8 interactions.
Protein family/group databases
MEROPS	M16.973. M16.981.
PTM databases
PhosphoSite	P31930.
2-D gel databases
SWISS-2DPAGE	P31930.
HSC-2DPAGE	P31930.
OGP	P31930.
PHCI-2DPAGE	P31930.
REPRODUCTION-2DPAGE	IPI00013847.
Siena-2DPAGE	P31930.
Proteomic databases
PeptideAtlas	P31930.
PRIDE	P31930.
Genome annotation databases
Ensembl	ENSG00000010256. Homo sapiens. [Contig view]
GeneID	7384.
KEGG	hsa:7384.
NMPDR	fig|9606.3.peg.22483.
Organism-specific databases
GeneCards	GC03M048611.
H-InvDB	HIX0003290.
HGNC	HGNC:12585. UQCRC1.
HPA	HPA002815. HPA003525.
MIM	191328. gene.
PharmGKB	PA37216.
GenAtlas	Search...
Phylogenomic databases
HOGENOM	P31930.
HOVERGEN	P31930.
OMA	P31930. KHFSSIS.
Enzyme and pathway databases
Reactome	REACT_6305. Electron Transport Chain.
Gene expression databases
ArrayExpress	P31930.
Bgee	P31930.
CleanEx	HS_UQCRC1.
GermOnline	ENSG00000010256. Homo sapiens.
Family and domain databases
InterPro	IPR011237. Pept_M16_core. IPR011765. Pept_M16_N. IPR001431. Pept_M16_Zn_BS. IPR007863. Peptidase_M16_C. [Graphical view]
Gene3D	G3DSA:3.30.830.10. Pept_M16_core. 2 hits.
Pfam	PF00675. Peptidase_M16. 1 hit. PF05193. Peptidase_M16_C. 1 hit. [Graphical view]
PROSITE	PS00143. INSULINASE. False negative. [Graphical view]
ProtoNet	Search...
Other Resources
DrugBank	DB01117. Atovaquone.
NextBio	28912.
SOURCE	Search...

Entry information

Entry name

QCR1_HUMAN

Accession

Primary (citable) accession number: P31930
Secondary accession number(s): Q96DD2

Entry history

Integrated into UniProtKB/Swiss-Prot:	July 1, 1993
Last sequence update:	April 4, 2006
Last modified:	June 16, 2009
This is version 100 of the entry and version 3 of the sequence. [Complete history]

Entry status

Reviewed (UniProtKB/Swiss-Prot)

Annotation project

HPI (Human Proteome Initiative)