|Ubiquinol-cytochrome c reductase binding protein|
|Symbols||; MC3DN3; QCR7; QP-C; QPC; UQBC; UQBP; UQCR6; UQPC|
|External IDs||ChEMBL: GeneCards:|
The ubiquinone-binding protein is a nucleus-encoded component of ubiquinol-cytochrome c oxidoreductase (Complex III; EC 184.108.40.206) in the mitochondrial respiratory chain and plays an important role in electron transfer as a complex of ubiquinone and QP-C. Complex III consists of 10 nuclear-encoded subunits and 1 mitochondrial-encoded subunit.
and ten nuclear genes:
- UQCRC1, UQCRC2, Cytochrome C1, UQCRFS1 (Rieske protein), UQCRB (this protein), UQCRQ ("11kDa protein"), UQCRH (cyt c1 Hinge protein), UCRC ("cyt. c1 associated protein"), and UQCR ("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 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 going from Laemli gels to Weber&Osborne gels, and one might suspect the name "Q-binding protein" arose from confusion with subunit 7. I have been told however that this is not the case, and both proteins were separately identified as Q-binding proteins. Genome anotators improved the situation by naming its gene "UQCR binding", UQCRB.
- Suzuki H, Hosokawa Y, Toda H, et al. (1990). "Common protein-binding sites in the 5'-flanking regions of human genes for cytochrome c1 and ubiquinone-binding protein.". J. Biol. Chem. 265 (14): 8159–63. PMID 2159470.
- Hosokawa Y, Suzuki H, Nishikimi M, et al. (1990). "Chromosomal assignment of the gene for the ubiquinone-binding protein of human mitochondrial cytochrome bc1 complex.". Biochem. Int. 21 (1): 41–4. PMID 2167087.
- Suzuki H, Hosokawa Y, Toda H, et al. (1989). "Isolation of a single nuclear gene encoding human ubiquinone-binding protein in complex III of mitochondrial respiratory chain.". Biochem. Biophys. Res. Commun. 161 (1): 371–8. doi:10.1016/0006-291X(89)91607-0. PMID 2543413.
- Wakabayashi S, Takao T, Shimonishi Y, et al. (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.". J. Biol. Chem. 260 (1): 337–43. PMID 2981208.
- Suzuki H, Hosokawa Y, Toda H, et al. (1988). "Cloning and sequencing of a cDNA for human mitochondrial ubiquinone-binding protein of complex III.". Biochem. Biophys. Res. Commun. 156 (2): 987–94. doi:10.1016/S0006-291X(88)80941-0. PMID 3056408.
- Malaney S, Heng HH, Tsui LC, et al. (1996). "Localization of the human gene encoding the 13.3-kDa subunit of mitochondrial complex III (UQCRB) to 8q22 by in situ hybridization.". Cytogenet. Cell Genet. 73 (4): 297–9. doi:10.1159/000134360. PMID 8751380.
- Strausberg RL, Feingold EA, Grouse LH, et al. (2003). "Generation and initial analysis of more than 15,000 full-length human and mouse cDNA sequences.". Proc. Natl. Acad. Sci. U.S.A. 99 (26): 16899–903. doi:10.1073/pnas.242603899. PMC 139241. PMID 12477932.
- Haut S, Brivet M, Touati G, et al. (2003). "A deletion in the human QP-C gene causes a complex III deficiency resulting in hypoglycaemia and lactic acidosis.". Hum. Genet. 113 (2): 118–22. doi:10.1007/s00439-003-0946-0. PMID 12709789.
- Gerhard DS, Wagner L, Feingold EA, et al. (2004). "The status, quality, and expansion of the NIH full-length cDNA project: the Mammalian Gene Collection (MGC).". Genome Res. 14 (10B): 2121–7. doi:10.1101/gr.2596504. PMC 528928. PMID 15489334.
- Rual JF, Venkatesan K, Hao T, et al. (2005). "Towards a proteome-scale map of the human protein-protein interaction network.". Nature 437 (7062): 1173–8. doi:10.1038/nature04209. PMID 16189514.
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