The world's first wiki where authorship really matters (Nature Genetics, 2008). Due credit and reputation for authors. Imagine a global collaborative knowledge base for original thoughts. Search thousands of articles and collaborate with scientists around the globe.

wikigene or wiki gene protein drug chemical gene disease author authorship tracking collaborative publishing evolutionary knowledge reputation system wiki2.0 global collaboration genes proteins drugs chemicals diseases compound
Hoffmann, R. A wiki for the life sciences where authorship matters. Nature Genetics (2008)



Gene Review

UQCRB  -  ubiquinol-cytochrome c reductase binding...

Homo sapiens

Synonyms: Complex III subunit 7, Complex III subunit VII, Cytochrome b-c1 complex subunit 7, MC3DN3, QCR7, ...
Welcome! If you are familiar with the subject of this article, you can contribute to this open access knowledge base by deleting incorrect information, restructuring or completely rewriting any text. Read more.

Disease relevance of UQCRB

  • A deletion in the human QP-C gene causes a complex III deficiency resulting in hypoglycaemia and lactic acidosis [1].

High impact information on UQCRB

  • However, in rat and mouse a synteny break resides approximately 70 kb upstream of Gdf6, such that Gdf6 and Uqcrb are on separate chromosomes [2].
  • We report here a deletion in the nuclear gene UQCRB encoding the human ubiquinone-binding protein of complex III (QP-C subunit or subunit VII) in a consanguineous family with an isolated complex III defect [1].
  • Low temperature (-196 degrees C) spectral studies performed on isolated mitochondria from cultured skin fibroblast of the proband showed a decreased cytochrome b content suggestive of a role for the QP-C subunit in the assembly or maintenance of complex III structure [1].
  • The ubiquinone-binding protein (QP-C) is a nuclear-encoded component of ubiquinol-cytochrome c oxidoreductase in the mitochondrial respiratory chain and plays an important role in electron transfer as a ubiquinone-QP-C complex [3].
  • This implies that the human QP-C is synthesized without a presequence which is required for import of most nuclear-encoded mitochondrial proteins into mitochondria [3].

Biological context of UQCRB


Anatomical context of UQCRB

  • We recently isolated a nuclear gene for human QP-C and used, in the present study, its fragment as a probe for Southern blot analysis of EcoRI-digested DNAs prepared from 14 human-mouse somatic cell hybrids [5].

Analytical, diagnostic and therapeutic context of UQCRB


  1. A deletion in the human QP-C gene causes a complex III deficiency resulting in hypoglycaemia and lactic acidosis. Haut, S., Brivet, M., Touati, G., Rustin, P., Lebon, S., Garcia-Cazorla, A., Saudubray, J.M., Boutron, A., Legrand, A., Slama, A. Hum. Genet. (2003) [Pubmed]
  2. Comparative sequence analysis of the Gdf6 locus reveals a duplicon-mediated chromosomal rearrangement in rodents and rapidly diverging coding and regulatory sequences. Mortlock, D.P., Portnoy, M.E., Chandler, R.L., Green, E.D. Genomics (2004) [Pubmed]
  3. Cloning and sequencing of a cDNA for human mitochondrial ubiquinone-binding protein of complex III. Suzuki, H., Hosokawa, Y., Toda, H., Nishikimi, M., Ozawa, T. Biochem. Biophys. Res. Commun. (1988) [Pubmed]
  4. Localization of the human gene encoding the 13.3-kDa subunit of mitochondrial complex III (UQCRB) to 8q22 by in situ hybridization. Malaney, S., Heng, H.H., Tsui, L.C., Shi, X.M., Robinson, B.H. Cytogenet. Cell Genet. (1996) [Pubmed]
  5. Chromosomal assignment of the gene for the ubiquinone-binding protein of human mitochondrial cytochrome bc1 complex. Hosokawa, Y., Suzuki, H., Nishikimi, M., Matsukage, A., Yoshida, M.C., Ozawa, T. Biochem. Int. (1990) [Pubmed]
WikiGenes - Universities