P22695 (QCR2_HUMAN) Reviewed, UniProtKB/Swiss-Prot

Names and origin

Protein names

Recommended name:
Cytochrome b-c1 complex subunit 2, mitochondrial

Alternative name(s):
Complex III subunit 2
Core protein II
Ubiquinol-cytochrome-c reductase complex core protein 2

Gene names

Name:

UQCRC2

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	453 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. The core protein 2 is required for the assembly of the complex.
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; Peripheral membrane protein; Matrix side.
Sequence similarities	Belongs to the peptidase M16 family. UQCRC2/QCR2 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
Technical term	Complete proteome Direct protein sequencing Reference proteome
Gene Ontology (GO)
Biological_process	aerobic respiration Traceable author statement PubMed 8288258. Source: ProtInc oxidative phosphorylation Traceable author statement PubMed 8288258. Source: ProtInc proteolysis Inferred from electronic annotation. Source: InterPro respiratory electron transport chain Traceable author statement. Source: Reactome small molecule metabolic process Traceable author statement. Source: Reactome
Cellular_component	mitochondrial inner membrane Traceable author statement. Source: Reactome mitochondrial respiratory chain complex III Inferred from electronic annotation. Source: Compara
Molecular_function	metal ion binding Inferred from electronic annotation. Source: InterPro metalloendopeptidase activity Inferred from electronic annotation. Source: InterPro
Complete GO annotation...

Binary interactions

With	Entry	#Exp.	IntAct	Notes
CACNA1A	O00555	2	EBI-1051424,EBI-766279

Sequence annotation (Features)

	Feature key	Position(s)	Length	Description	Graphical view	Feature identifier
Molecule processing
	Transit peptide	1 – 14	14	Mitochondrion
	Chain	15 – 453	439	Cytochrome b-c1 complex subunit 2, mitochondrial		PRO_0000026791
Amino acid modifications
	Modified residue	159	1	N6-acetyllysine By similarity
	Modified residue	250	1	N6-acetyllysine By similarity
Natural variations
	Natural variant	148	1	R → S. Corresponds to variant rs2228473 [ dbSNP | Ensembl ].		VAR_029336
	Natural variant	183	1	R → Q. Ref.1 Corresponds to variant rs4850 [ dbSNP | Ensembl ].		VAR_034582
	Natural variant	208	1	F → Y in a colorectal cancer sample; somatic mutation. Ref.7		VAR_036479
	Natural variant	254	1	R → H. Corresponds to variant rs11863893 [ dbSNP | Ensembl ].		VAR_034583
Experimental info
	Sequence conflict	360	1	T → R in AAA35710. Ref.1

Sequences

	Sequence		Length	Mass (Da)
	P22695 [UniParc]. Last modified July 11, 2002. Version 3. Checksum: BFA655C24C07AF52	FASTA	453	48,443
	10 20 30 40 50 60 MKLLTRAGSF SRFYSLKVAP KVKATAAPAG APPQPQDLEF TKLPNGLVIA SLENYSPVSR 70 80 90 100 110 120 IGLFIKAGSR YEDFSNLGTT HLLRLTSSLT TKGASSFKIT RGIEAVGGKL SVTATRENMA 130 140 150 160 170 180 YTVECLRGDV DILMEFLLNV TTAPEFRRWE VADLQPQLKI DKAVAFQNPQ THVIENLHAA 190 200 210 220 230 240 AYRNALANPL YCPDYRIGKV TSEELHYFVQ NHFTSARMAL IGLGVSHPVL KQVAEQFLNM 250 260 270 280 290 300 RGGLGLSGAK ANYRGGEIRE QNGDSLVHAA FVAESAVAGS AEANAFSVLQ HVLGAGPHVK 310 320 330 340 350 360 RGSNTTSHLH QAVAKATQQP FDVSAFNASY SDSGLFGIYT ISQATAAGDV IKAAYNQVKT 370 380 390 400 410 420 IAQGNLSNTD VQAAKNKLKA GYLMSVESSE CFLEEVGSQA LVAGSYMPPS TVLQQIDSVA 430 440 450 NADIINAAKK FVSGQKSMAA SGNLGHTPFV DEL

References

	« Hide 'large scale' referencesShow 'large scale' references »
[1]	"Complementary DNA encoding core protein II of human mitochondrial cytochrome bc1 complex. Substantial diversity in deduced primary structure from its yeast counterpart." Hosokawa Y., Suzuki H., Toda H., Nishikimi M., Ozawa T. J. Biol. Chem. 264:13483-13488(1989) [PubMed] [Europe PMC] [Abstract] Cited for: NUCLEOTIDE SEQUENCE [MRNA], VARIANT GLN-183.
[2]	"Complete sequencing and characterization of 21,243 full-length human cDNAs." Ota T., Suzuki Y., Nishikawa T., Otsuki T., Sugiyama T., Irie R., Wakamatsu A., Hayashi K., Sato H., Nagai K., Kimura K., Makita H., Sekine M., Obayashi M., Nishi T., Shibahara T., Tanaka T., Ishii S. , Yamamoto J., Saito K., Kawai Y., Isono Y., Nakamura Y., Nagahari K., Murakami K., Yasuda T., Iwayanagi T., Wagatsuma M., Shiratori A., Sudo H., Hosoiri T., Kaku Y., Kodaira H., Kondo H., Sugawara M., Takahashi M., Kanda K., Yokoi T., Furuya T., Kikkawa E., Omura Y., Abe K., Kamihara K., Katsuta N., Sato K., Tanikawa M., Yamazaki M., Ninomiya K., Ishibashi T., Yamashita H., Murakawa K., Fujimori K., Tanai H., Kimata M., Watanabe M., Hiraoka S., Chiba Y., Ishida S., Ono Y., Takiguchi S., Watanabe S., Yosida M., Hotuta T., Kusano J., Kanehori K., Takahashi-Fujii A., Hara H., Tanase T.-O., Nomura Y., Togiya S., Komai F., Hara R., Takeuchi K., Arita M., Imose N., Musashino K., Yuuki H., Oshima A., Sasaki N., Aotsuka S., Yoshikawa Y., Matsunawa H., Ichihara T., Shiohata N., Sano S., Moriya S., Momiyama H., Satoh N., Takami S., Terashima Y., Suzuki O., Nakagawa S., Senoh A., Mizoguchi H., Goto Y., Shimizu F., Wakebe H., Hishigaki H., Watanabe T., Sugiyama A., Takemoto M., Kawakami B., Yamazaki M., Watanabe K., Kumagai A., Itakura S., Fukuzumi Y., Fujimori Y., Komiyama M., Tashiro H., Tanigami A., Fujiwara T., Ono T., Yamada K., Fujii Y., Ozaki K., Hirao M., Ohmori Y., Kawabata A., Hikiji T., Kobatake N., Inagaki H., Ikema Y., Okamoto S., Okitani R., Kawakami T., Noguchi S., Itoh T., Shigeta K., Senba T., Matsumura K., Nakajima Y., Mizuno T., Morinaga M., Sasaki M., Togashi T., Oyama M., Hata H., Watanabe M., Komatsu T., Mizushima-Sugano J., Satoh T., Shirai Y., Takahashi Y., Nakagawa K., Okumura K., Nagase T., Nomura N., Kikuchi H., Masuho Y., Yamashita R., Nakai K., Yada T., Nakamura Y., Ohara O., Isogai T., Sugano S. Nat. Genet. 36:40-45(2004) [PubMed] [Europe PMC] [Abstract] Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE MRNA]. Tissue: Uterus.
[3]	Mural R.J., Istrail S., Sutton G.G., Florea L., Halpern A.L., Mobarry C.M., Lippert R., Walenz B., Shatkay H., Dew I., Miller J.R., Flanigan M.J., Edwards N.J., Bolanos R., Fasulo D., Halldorsson B.V., Hannenhalli S., Turner R. , Yooseph S., Lu F., Nusskern D.R., Shue B.C., Zheng X.H., Zhong F., Delcher A.L., Huson D.H., Kravitz S.A., Mouchard L., Reinert K., Remington K.A., Clark A.G., Waterman M.S., Eichler E.E., Adams M.D., Hunkapiller M.W., Myers E.W., Venter J.C. Submitted (JUL-2005) to the EMBL/GenBank/DDBJ databases Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE GENOMIC DNA].
[4]	"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]. Tissue: Lung and Skin.
[5]	Lubec G., Vishwanath V. Submitted (MAR-2007) to UniProtKB Cited for: PROTEIN SEQUENCE OF 43-60; 71-84 AND 200-217, MASS SPECTROMETRY. Tissue: Brain and Cajal-Retzius cell.
[6]	"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].
[7]	"The consensus coding sequences of human breast and colorectal cancers." Sjoeblom T., Jones S., Wood L.D., Parsons D.W., Lin J., Barber T.D., Mandelker D., Leary R.J., Ptak J., Silliman N., Szabo S., Buckhaults P., Farrell C., Meeh P., Markowitz S.D., Willis J., Dawson D., Willson J.K.V. , Gazdar A.F., Hartigan J., Wu L., Liu C., Parmigiani G., Park B.H., Bachman K.E., Papadopoulos N., Vogelstein B., Kinzler K.W., Velculescu V.E. Science 314:268-274(2006) [PubMed] [Europe PMC] [Abstract] Cited for: VARIANT [LARGE SCALE ANALYSIS] TYR-208.
+	Additional computationally mapped references.

Cross-references

Sequence databases
EMBL GenBank DDBJ	J04973 mRNA. Translation: AAA35710.1. AK094006 mRNA. Translation: BAG52796.1. CH471249 Genomic DNA. Translation: EAW50592.1. BC003136 mRNA. Translation: AAH03136.1. BC000484 mRNA. Translation: AAH00484.1.
IPI	IPI00305383.
PIR	A32629.
RefSeq	NP_003357.2. NM_003366.2.
UniGene	Hs.528803.
3D structure databases
ProteinModelPortal	P22695.
ModBase	Search...
Protein-protein interaction databases
IntAct	P22695. 5 interactions.
STRING	9606.ENSP00000268379.
Protein family/group databases
MEROPS	M16.974.
PTM databases
PhosphoSite	P22695.
Polymorphism databases
DMDM	21903482.
2D gel databases
REPRODUCTION-2DPAGE	IPI00305383.
UCD-2DPAGE	P22695.
Proteomic databases
PaxDb	P22695.
PeptideAtlas	P22695.
PRIDE	P22695.
Protocols and materials databases
DNASU	7385.
StructuralBiologyKnowledgebase	Search...
Genome annotation databases
Ensembl	ENST00000268379; ENSP00000268379; ENSG00000140740.
GeneID	7385.
KEGG	hsa:7385.
UCSC	uc002djx.3. human.
Organism-specific databases
CTD	7385.
GeneCards	GC16P021963.
HGNC	HGNC:12586. UQCRC2.
HPA	HPA007998. HPA019146.
MIM	191329. gene.
neXtProt	NX_P22695.
PharmGKB	PA37217.
GenAtlas	Search...
Phylogenomic databases
eggNOG	COG0612.
HOGENOM	HOG000046923.
HOVERGEN	HBG055236.
InParanoid	P22695.
KO	K00415.
OMA	FVSGQKS.
OrthoDB	EOG4QRH45.
PhylomeDB	P22695.
Enzyme and pathway databases
Reactome	REACT_111217. Metabolism.
Gene expression databases
ArrayExpress	P22695.
Bgee	P22695.
CleanEx	HS_UQCRC2.
Genevestigator	P22695.
GermOnline	ENSG00000140740. Homo sapiens.
Family and domain databases
Gene3D	3.30.830.10. 2 hits.
InterPro	IPR011249. Metalloenz_LuxS/M16. IPR011237. Pept_M16_dom. IPR011765. Pept_M16_N. IPR001431. Pept_M16_Zn_BS. IPR007863. Peptidase_M16_C. [Graphical view]
Pfam	PF00675. Peptidase_M16. 1 hit. PF05193. Peptidase_M16_C. 1 hit. [Graphical view]
SUPFAM	SSF63411. Metalloenz_metal-bd. 2 hits.
PROSITE	PS00143. INSULINASE. 1 hit. [Graphical view]
ProtoNet	Search...
Other
ChiTaRS	UQCRC2. human.
GenomeRNAi	7385.
NextBio	28916.
SOURCE	Search...

Entry information

Entry name

QCR2_HUMAN

Accession

Primary (citable) accession number: P22695
Secondary accession number(s): B3KSN4, Q9BQ05

Entry history

Integrated into UniProtKB/Swiss-Prot:	August 1, 1991
Last sequence update:	July 11, 2002
Last modified:	May 1, 2013
This is version 135 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.