Gene Symbol: Uqcrfs1
Description: ubiquinol-cytochrome c reductase, Rieske iron-sulfur polypeptide 1
Alias: 4430402G14Rik, AI875505, cytochrome b-c1 complex subunit Rieske, mitochondrial, RISP, Rieske protein UQCRFS1, complex III subunit 5, cytochrome b-c1 complex subunit 5, rieske iron-sulfur protein, ubiquinol-cytochrome c reductase iron-sulfur subunit
Species: mouse
Products:     Uqcrfs1

Top Publications

  1. Schilling B, Murray J, Yoo C, Row R, Cusack M, Capaldi R, et al. Proteomic analysis of succinate dehydrogenase and ubiquinol-cytochrome c reductase (Complex II and III) isolated by immunoprecipitation from bovine and mouse heart mitochondria. Biochim Biophys Acta. 2006;1762:213-22 pubmed
    ..The identification of several posttranslational modifications, including the covalent FAD modification of flavoprotein subunit 1 from Complex II, was possible due to high mass spectrometric sequence coverage. ..
  2. Garcia S, Diaz F, Moraes C. A 3' UTR modification of the mitochondrial rieske iron sulfur protein in mice produces a specific skin pigmentation phenotype. J Invest Dermatol. 2008;128:2343-5 pubmed publisher
  3. Diaz F, Garcia S, Padgett K, Moraes C. A defect in the mitochondrial complex III, but not complex IV, triggers early ROS-dependent damage in defined brain regions. Hum Mol Genet. 2012;21:5066-77 pubmed publisher
    ..These conditional knockouts (cKOs) were created by ablation of the genes coding for the Rieske iron-sulfur protein (RISP) and COX10, respectively...
  4. Waypa G, Marks J, Guzy R, Mungai P, Schriewer J, Dokic D, et al. Superoxide generated at mitochondrial complex III triggers acute responses to hypoxia in the pulmonary circulation. Am J Respir Crit Care Med. 2013;187:424-32 pubmed publisher
    ..A mouse permitting Cre-mediated conditional deletion of the Rieske iron-sulfur protein (RISP) of Complex III was generated...
  5. Bottani E, Cerutti R, Harbour M, Ravaglia S, Doğan S, Giordano C, et al. TTC19 Plays a Husbandry Role on UQCRFS1 Turnover in the Biogenesis of Mitochondrial Respiratory Complex III. Mol Cell. 2017;67:96-105.e4 pubmed publisher
    ..e., after the incorporation of the iron-sulfur Rieske protein (UQCRFS1). The in situ maturation of UQCRFS1 produces N-terminal polypeptides, which remain bound to holocomplex III...
  6. Hilander T, Zhou X, Konovalova S, Zhang F, Euro L, Chilov D, et al. Editing activity for eliminating mischarged tRNAs is essential in mammalian mitochondria. Nucleic Acids Res. 2018;46:849-860 pubmed publisher
    ..These results indicate that tRNA proofreading is essential in mammalian mitochondria, and cannot be overcome by other quality control mechanisms. ..
  7. Diaz F, Enriquez J, Moraes C. Cells lacking Rieske iron-sulfur protein have a reactive oxygen species-associated decrease in respiratory complexes I and IV. Mol Cell Biol. 2012;32:415-29 pubmed publisher
    ..the assembly/stability of respiratory complexes in mouse lung fibroblasts lacking the Rieske iron-sulfur protein (RISP knockout [KO]cells), one of the catalytic subunits of CIII...
  8. Wiley S, Murphy A, Ross S, van der Geer P, Dixon J. MitoNEET is an iron-containing outer mitochondrial membrane protein that regulates oxidative capacity. Proc Natl Acad Sci U S A. 2007;104:5318-23 pubmed
    ..Cardiac mitochondria isolated from mitoNEET-null mice demonstrate a reduced oxidative capacity, suggesting that mito- NEET is an important iron-containing protein involved in the control of maximal mitochondrial respiratory rates. ..
  9. Ghezzi D, Arzuffi P, Zordan M, Da Re C, Lamperti C, Benna C, et al. Mutations in TTC19 cause mitochondrial complex III deficiency and neurological impairment in humans and flies. Nat Genet. 2011;43:259-63 pubmed publisher
    ..TTC19 is a putative cIII assembly factor whose disruption is associated with severe neurological abnormalities in humans and flies. ..

More Information


  1. Hughes B, Hekimi S. A mild impairment of mitochondrial electron transport has sex-specific effects on lifespan and aging in mice. PLoS ONE. 2011;6:e26116 pubmed publisher
    ..For example, disruption of the function of the 'Rieske' iron-sulfur protein (RISP) of complex III of the mitochondrial electron transport chain can result in increased lifespan in the nematode worm ..
  2. Sena L, Li S, Jairaman A, Prakriya M, Ezponda T, Hildeman D, et al. Mitochondria are required for antigen-specific T cell activation through reactive oxygen species signaling. Immunity. 2013;38:225-36 pubmed publisher
    ..These mice could not induce antigen-specific expansion of T cells in vivo, but Uqcrfs1(-/-) T cells retained the ability to proliferate in vivo under lymphopenic conditions...
  3. Lapointe J, Hughes B, Bigras E, Hekimi S. Compensatory elevation of voluntary activity in mouse mutants with impaired mitochondrial energy metabolism. Physiol Rep. 2014;2: pubmed publisher
    ..for heterozygous mice carrying a mutation for the gene encoding the "Rieske" protein of mitochondrial complex III (RISP(+/P224S))...