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Aliases UQCRB, MC3DN3, QCR7, QP-C, QPC, UQBC, UQBP, UQCR6, UQPC, ubiquinol-cytochrome c reductase binding protein
External IDs MGI: 1914780 HomoloGene: 38164 GeneCards: UQCRB
Gene location (Human)
Chromosome 8 (human)
Chr. Chromosome 8 (human)[1]
Chromosome 8 (human)
Genomic location for UQCRB
Genomic location for UQCRB
Band 8q22.1 Start 96,225,920 bp[1]
End 96,235,634 bp[1]
Species Human Mouse
RefSeq (mRNA)



RefSeq (protein)



Location (UCSC) Chr 8: 96.23 – 96.24 Mb Chr 13: 66.9 – 66.91 Mb
PubMed search [3] [4]
View/Edit Human View/Edit Mouse

Ubiquinol-cytochrome c reductase binding protein, also known as UQCRB, is a protein which in humans is encoded by the UQCRB gene.[5]


The gene product of UQCRB is a subunit of the respiratory chain protein Ubiquinol Cytochrome c Reductase (UQCR, Complex III or Cytochrome bc1 complex; E.C., which consists of the products of one mitochondrially encoded gene, MTCYTB (mitochondrial cytochrome b) and ten nuclear genes: UQCRC1, UQCRC2, Cytochrome c1, UQCRFS1 (Rieske protein), UQCRB, "14kDa protein", UQCRH (cyt c1 Hinge protein), Rieske Protein presequence, "cyt. c1 associated protein", and "Rieske-associated protein". After processing, the cleaved leader sequence of the iron-sulfur protein is retained as subunit 9, giving 11 subunits from 10 genes.


The ubiquinone-binding protein is a nucleus-encoded component of ubiquinol-cytochrome c oxidoreductase (Complex III; EC in the mitochondrial respiratory chain and plays an important role in electron transfer as a complex of ubiquinone and QP-C.[5]

The bovine gene product (subunit 6) was sequenced under the name "ubiquinone-binding protein", however there is little or no evidence for a role in ubiquinone binding. Subunit 7 was identified as a Q-binding protein by photo-labeling with a ubiquinone analog (subsequent structures show it to be exposed to the lipid phase but not involved in either Q-binding site). Subunits 6 and 7 reverse position on transfer from Laemli gels to Weber&Osborne gels, and one might suspect the name "Q-binding protein" arose from confusion with subunit 7. However, it has been claimed that both proteins were separately identified as Q-binding proteins. Genome annotators improved the situation by naming this gene "UQCR binding", or UQCRB.


Further reading[edit]

  • Chang J, Jung HJ, Park HJ, Cho SW, Lee SK, Kwon HJ (Sep 2015). "Cell-permeable mitochondrial ubiquinol-cytochrome c reductase binding protein induces angiogenesis in vitro and in vivo". Cancer Lett. 366 (1): 52–60. doi:10.1016/j.canlet.2015.06.013. PMID 26118773. 
  • Jung HJ, Cho M, Kim Y, Han G, Kwon HJ (Oct 2014). "Development of a novel class of mitochondrial ubiquinol-cytochrome c reductase binding protein (UQCRB) modulators as promising antiangiogenic leads". J Med Chem. 57 (19): 7990–8. doi:10.1021/jm500863j. PMID 25244355. 
  • Chang J, Jung HJ, Jeong SH, Kim HK, Han J, Kwon HJ (Dec 2014). "A mutation in the mitochondrial protein UQCRB promotes angiogenesis through the generation of mitochondrial reactive oxygen species". Biochem Biophys Res Commun. 455 (3–4): 290–7. doi:10.1016/j.bbrc.2014.11.005. PMID 25446085. 
  • Cho YS, Jung HJ, Seok SH, Payumo AY, Chen JK, Kwon HJ (Apr 2013). "Functional inhibition of UQCRB suppresses angiogenesis in zebrafish". Biochem Biophys Res Commun. 433 (4): 396–400. doi:10.1016/j.bbrc.2013.02.082. PMC 3691074Freely accessible. PMID 23454382. 
  • Jung HJ, Kwon HJ (May 2013). "Exploring the role of mitochondrial UQCRB in angiogenesis using small molecules". Mol Biosyst. 9 (5): 930–9. doi:10.1039/c3mb25426g. PMID 23475074. 
  • Jung HJ, Kim KH, Kim ND, Han G, Kwon HJ (Feb 2011). "Identification of a novel small molecule targeting UQCRB of mitochondrial complex III and its anti-angiogenic activity". Bioorg Med Chem Lett. 21 (3): 1052–6. doi:10.1016/j.bmcl.2010.12.002. PMID 21215626. 
  • Jung HJ, Kim Y, Chang J, Kang SW, Kim JH, Kwon HJ (May 2013). "Mitochondrial UQCRB regulates VEGFR2 signaling in endothelial cells". J Mol Med (Berl). 91 (9): 1117–28. doi:10.1007/s00109-013-1049-6. PMID 23708980. 
  • Suzuki H, Hosokawa Y, Toda H, Nishikimi M, Ozawa T (May 1990). "Common protein-binding sites in the 5'-flanking regions of human genes for cytochrome c1 and ubiquinone-binding protein". The Journal of Biological Chemistry. 265 (14): 8159–63. PMID 2159470. 
  • Hosokawa Y, Suzuki H, Nishikimi M, Matsukage A, Yoshida MC, Ozawa T (1990). "Chromosomal assignment of the gene for the ubiquinone-binding protein of human mitochondrial cytochrome bc1 complex". Biochemistry International. 21 (1): 41–4. PMID 2167087. 
  • Suzuki H, Hosokawa Y, Toda H, Nishikimi M, Ozawa T (May 1989). "Isolation of a single nuclear gene encoding human ubiquinone-binding protein in complex III of mitochondrial respiratory chain". Biochemical and Biophysical Research Communications. 161 (1): 371–8. doi:10.1016/0006-291X(89)91607-0. PMID 2543413. 
  • Wakabayashi S, Takao T, Shimonishi Y, Kuramitsu S, Matsubara H, Wang T, Zhang Z, King TE (Jan 1985). "Complete amino acid sequence of the ubiquinone binding protein (QP-C), a protein similar to the 14,000-dalton subunit of the yeast ubiquinol-cytochrome c reductase complex". The Journal of Biological Chemistry. 260 (1): 337–43. PMID 2981208. 
  • Suzuki H, Hosokawa Y, Toda H, Nishikimi M, Ozawa T (Oct 1988). "Cloning and sequencing of a cDNA for human mitochondrial ubiquinone-binding protein of complex III". Biochemical and Biophysical Research Communications. 156 (2): 987–94. doi:10.1016/S0006-291X(88)80941-0. PMID 3056408. 
  • Malaney S, Heng HH, Tsui LC, Shi XM, Robinson BH (1996). "Localization of the human gene encoding the 13.3-kDa subunit of mitochondrial complex III (UQCRB) to 8q22 by in situ hybridization". Cytogenetics and Cell Genetics. 73 (4): 297–9. doi:10.1159/000134360. PMID 8751380. 
  • Haut S, Brivet M, Touati G, Rustin P, Lebon S, Garcia-Cazorla A, Saudubray JM, Boutron A, Legrand A, Slama A (Jul 2003). "A deletion in the human QP-C gene causes a complex III deficiency resulting in hypoglycaemia and lactic acidosis". Human Genetics. 113 (2): 118–22. doi:10.1007/s00439-003-0946-0. PMID 12709789. 
  • Rual JF, Venkatesan K, Hao T, Hirozane-Kishikawa T, Dricot A, Li N, Berriz GF, Gibbons FD, Dreze M, Ayivi-Guedehoussou N, Klitgord N, Simon C, Boxem M, Milstein S, Rosenberg J, Goldberg DS, Zhang LV, Wong SL, Franklin G, Li S, Albala JS, Lim J, Fraughton C, Llamosas E, Cevik S, Bex C, Lamesch P, Sikorski RS, Vandenhaute J, Zoghbi HY, Smolyar A, Bosak S, Sequerra R, Doucette-Stamm L, Cusick ME, Hill DE, Roth FP, Vidal M (Oct 2005). "Towards a proteome-scale map of the human protein-protein interaction network". Nature. 437 (7062): 1173–8. doi:10.1038/nature04209. PMID 16189514. 

This article incorporates text from the United States National Library of Medicine, which is in the public domain.