UQCR11 (ubiquinol-cytochrome c reductase, complex III sub-unit XI) is a protein that in humans is encoded by the UQCR11 gene.[1] [2] UQCR11 is the smallest known component of Complex III in the mitochondrial respiratory chain.[2]
The UQCR11 gene, located on the p arm of chromosome 19 in position 13.3, is made up of 3 exons and is 8,329 base pairs in length.[2] The UQCR11 protein weighs 6.6 kDa and is composed of 56 amino acids.[3] [4] This gene encodes the smallest known component of the ubiquinol-cytochrome c reductase complex, which is also known as Complex III and is part of the mitochondrial respiratory chain.[2] In vertebrates, Complex III contains 11 sub-units: 3 respiratory sub-units, 2 core proteins and 6 low-molecular weight proteins.[5] [6] Proteobacterial complexes may contain as few as three sub-units.[7]
The UQCR11 protein may function as a binding factor for the iron-sulfur protein in Complex III, which is ubiquitous in human cells.[2] Complex III catalyzes the chemical reaction
QH2 + 2 ferricytochrome c
\rightleftharpoons
Thus, the two substrates of Complex III are dihydroquinone (QH2) and ferri- (Fe3+) cytochrome c, whereas its 3 products are quinone (Q), ferro- (Fe2+) cytochrome c, and H+. This complex belongs to the family of oxidoreductases, specifically those acting on diphenols and related substances as donor with a cytochrome as acceptor. This enzyme participates in oxidative phosphorylation. It has four cofactors: cytochrome c1, cytochrome b-562, cytochrome b-566 and a 2-Iron ferredoxin of the Rieske type.