UQCRFS1

Ubiquinol-cytochrome c reductase, Rieske iron-sulfur polypeptide 1
PDB rendering based on 1be3.
Available structures PDB Ortholog search: PDBe, RCSB List of PDB id codes 1be3​, 1bgy​, 1l0l​, 1l0n​, 1ntk​, 1ntm​, 1ntz​, 1nu1​, 1pp9​, 1ppj​, 1qcr​, 1rie​, 1sqb​, 1sqp​, 1sqq​, 1sqv​, 1sqx​, 2a06​, 2fyu​, 2ibz​, 2qjk​, 2qjp​, 2qjy​, 3cwb​, 3cx5​, 3cxh​, 3h1h​, 3h1i​, 3h1j​, 3h1k​, 3h1l​, 3l70​, 3l71​, 3l72​, 3l73​, 3l74​, 3l75​
Identifiers
Symbols	UQCRFS1 ; RIP1; RIS1; RISP; UQCR5
External IDs	OMIM: 191327 MGI: 1913944 HomoloGene: 4378 GeneCards: UQCRFS1 Gene
EC number	1.10.2.2
Gene ontology Molecular function • ubiquinol-cytochrome-c reductase activity • protein complex binding • metal ion binding • 2 iron, 2 sulfur cluster binding Cellular component • mitochondrion • mitochondrial inner membrane • mitochondrial respiratory chain complex III • myelin sheath Biological process • response to hormone • respiratory electron transport chain • response to drug • cellular metabolic process • small molecule metabolic process • response to antibiotic • hydrogen ion transmembrane transport Sources: Amigo / QuickGO
RNA expression pattern
More reference expression data
Orthologs
Species	Human	Mouse
Entrez	7386	66694
Ensembl	ENSG00000169021	ENSMUSG00000038462
UniProt	P47985	Q9CR68
RefSeq (mRNA)	NM_006003	NM_025710
RefSeq (protein)	NP_005994	NP_079986
Location (UCSC)	Chr 19: 29.21 – 29.21 Mb	Chr 13: 30.54 – 30.55 Mb
PubMed search

Ubiquinol-cytochrome c reductase, Rieske iron-sulfur polypeptide 1, also known as UQCRFS1, is a protein which in humans is encoded by the UQCRFS1 gene.^[1]

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").

After processing the cleaved leader sequence of the iron-sulfur protein is retained as subunit 9, giving 11 subunits from 10 genes.

References

1. "Entrez Gene: UQCRFS1 ubiquinol-cytochrome c reductase, Rieske iron-sulfur polypeptide 1".

Further reading

• Nishikimi M, Hosokawa Y, Toda H, et al. (1990). "The primary structure of human Rieske iron-sulfur protein of mitochondrial cytochrome bc1 complex deduced from cDNA analysis.". Biochem. Int. 20 (1): 155–60. PMID 2158323.
• Shimomura Y, Nishikimi M, Ozawa T (1986). "Novel purification of cytochrome c1 from mitochondrial Complex III. Reconstitution of antimycin-insensitive electron transfer with the iron-sulfur protein and cytochrome c1.". J. Biol. Chem. 260 (28): 15075–80. PMID 2999105.
• Pennacchio LA, Bergmann A, Fukushima A; et al. (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. PMID 7721092.
• Duncan AM, Anderson L, Duff C, et al. (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. PMID 8088805.
• Sarto C, Marocchi A, Sanchez JC, et al. (1997). "Renal cell carcinoma and normal kidney protein expression.". Electrophoresis 18 (3–4): 599–604. doi:10.1002/elps.1150180343. PMID 9150947.
• 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.
• Kaneko SJ, Gerasimova T, Smith ST, et al. (2003). "CA125 and UQCRFS1 FISH studies of ovarian carcinoma". Gynecol. Oncol. 90 (1): 29–36. doi:10.1016/S0090-8258(03)00144-6. PMID 12821338.
• Ohashi Y, Kaneko SJ, Cupples TE, Young SR (2004). "Ubiquinol cytochrome c reductase (UQCRFS1) gene amplification in primary breast cancer core biopsy samples". Gynecol. Oncol. 93 (1): 54–8. doi:10.1016/j.ygyno.2004.01.019. PMID 15047214.
• Grimwood J, Gordon LA, Olsen A, et al. (2004). "The DNA sequence and biology of human chromosome 19". Nature 428 (6982): 529–35. doi:10.1038/nature02399. PMID 15057824.
• 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.