Ubiquinol-cytochrome c reductase, Rieske iron-sulfur polypeptide 1, also known as UQCRFS1, Rieske iron-sulfur (Fe-S) protein, Cytochrome b-c1 complex subunit 5, or Complex III subunit 5 is a protein which in humans is encoded by the UQCRFS1gene.[5] UQCRFS1 is a subunit of the respiratory chain protein Ubiquinol Cytochrome c Reductase (UQCR, Complex III or Cytochrome bc1 complex), which consists of the products of one mitochondrially encoded gene, MTCYTB (mitochondrial cytochrome b) and ten nuclear genes UQCRC1, UQCRC2, Cytochrome C1, UQCRFS1 (this protein, a type of Rieske protein), UQCRB,UQCRQ ("11kDa protein"), UQCRH (cyt c1 Hinge protein), UCRC ("cyt. c1 associated protein"), and UQCR ("Rieske-associated protein").[6]
Structure
UQCRFS1 is located on the q arm of chromosome 19 in position 12, has 2 exons, and spans 5,969 base pairs.[5] The UQCRFS1 gene produces a 29.7 kDa protein composed of 274 amino acids.[7][8] UQCRFS1 is a subunit of the respiratory chain protein Ubiquinol Cytochrome c Reductase (UQCR, Complex III or Cytochrome bc1 complex). The structure of the complex is a symmetric homodimer composed of one mitochondrial genome encoded cytochrome b subunit and ten other nucleus encoded subunits.[9] The primary structure of UQCRFS1 from cDNA analysis is composed of a 78 amino acid long N-terminal extension sequence.[10]
The UQCRFS1 gene has been shown to be involved in carcinogenesis of some cancers. It is mainly associated with more aggressive tumors, and results in the development of more aggressive phenotypes of breast cancers. The association was found with a grade 3 amplification of the UQCRFS1 gene.[13] In addition, Acute myeloid leukemia (AML) has been found to be associated with the amplification of UQCRFS1 gene.[14] In contrast, UQCRFS1 and complex III has been absent in renal cell carcinoma, though the mechanism is unknown.[15]
^Nishikimi M, Hosokawa Y, Toda H, Suzuki H, Ozawa T (1990). "The primary structure of human Rieske iron-sulfur protein of mitochondrial cytochrome bc1 complex deduced from cDNA analysis". Biochemistry International. 20 (1): 155–60. PMID2158323.
^Pennacchio LA, Bergmann A, Fukushima A, Okubo K, Salemi A, Lennon GG (April 1995). "Structure, sequence and location of the UQCRFS1 gene for the human Rieske Fe-S protein". Gene. 155 (2): 207–11. doi:10.1016/0378-1119(94)00683-j. PMID7721092.
^Ohashi Y, Kaneko SJ, Cupples TE, Young SR (April 2004). "Ubiquinol cytochrome c reductase (UQCRFS1) gene amplification in primary breast cancer core biopsy samples". Gynecologic Oncology. 93 (1): 54–8. doi:10.1016/j.ygyno.2004.01.019. PMID15047214.
^Sait SN, Qadir MU, Conroy JM, Matsui S, Nowak NJ, Baer MR (May 2002). "Double minute chromosomes in acute myeloid leukemia and myelodysplastic syndrome: identification of new amplification regions by fluorescence in situ hybridization and spectral karyotyping". Genes, Chromosomes & Cancer. 34 (1): 42–7. doi:10.1002/gcc.10038. PMID11921281.
^Sarto C, Marocchi A, Sanchez JC, Giannone D, Frutiger S, Golaz O, Wilkins MR, Doro G, Cappellano F, Hughes G, Hochstrasser DF, Mocarelli P (1997). "Renal cell carcinoma and normal kidney protein expression". Electrophoresis. 18 (3–4): 599–604. doi:10.1002/elps.1150180343. PMID9150947.
^
Kerrien S, Alam-Faruque Y, Aranda B, Bancarz I, Bridge A, Derow C, Dimmer E, Feuermann M, Friedrichsen A, Huntley R, Kohler C, Khadake J, Leroy C, Liban A, Lieftink C, Montecchi-Palazzi L, Orchard S, Risse J, Robbe K, Roechert B, Thorneycroft D, Zhang Y, Apweiler R, Hermjakob H (January 2007). "IntAct--open source resource for molecular interaction data". Nucleic Acids Research. 35 (Database issue): D561-5. doi:10.1093/nar/gkl958. PMC1751531. PMID17145710.
Further reading
Shimomura Y, Nishikimi M, Ozawa T (December 1985). "Novel purification of cytochrome c1 from mitochondrial Complex III. Reconstitution of antimycin-insensitive electron transfer with the iron-sulfur protein and cytochrome c1". The Journal of Biological Chemistry. 260 (28): 15075–80. PMID2999105.
Duncan AM, Anderson L, Duff C, Ozawa T, Suzuki H, Worton R, Rozen R (May 1994). "Assignment of the gene (UQCRFS1) for the Rieske iron-sulfur protein subunit of the mitochondrial cytochrome bc1 complex to the 22q13 and 19q12-q13.1 regions of the human genome". Genomics. 21 (1): 281–3. doi:10.1006/geno.1994.1260. PMID8088805.
Sarto C, Marocchi A, Sanchez JC, Giannone D, Frutiger S, Golaz O, Wilkins MR, Doro G, Cappellano F, Hughes G, Hochstrasser DF, Mocarelli P (1997). "Renal cell carcinoma and normal kidney protein expression". Electrophoresis. 18 (3–4): 599–604. doi:10.1002/elps.1150180343. PMID9150947.
Kaneko SJ, Gerasimova T, Smith ST, Lloyd KO, Suzumori K, Young SR (July 2003). "CA125 and UQCRFS1 FISH studies of ovarian carcinoma". Gynecologic Oncology. 90 (1): 29–36. doi:10.1016/S0090-8258(03)00144-6. PMID12821338.
Ohashi Y, Kaneko SJ, Cupples TE, Young SR (April 2004). "Ubiquinol cytochrome c reductase (UQCRFS1) gene amplification in primary breast cancer core biopsy samples". Gynecologic Oncology. 93 (1): 54–8. doi:10.1016/j.ygyno.2004.01.019. PMID15047214.