Gene Symbol: fh
Description: frataxin
Alias: BcDNA:AT09528, CG8971, DFH, Dfh, Dmel\CG8971, X25, dfh, frataxin, CG8971-PA, CG8971-PB, fh-PA, fh-PB, frataxin homolog, frataxin homologue, frataxin-like
Species: fruit fly

Top Publications

  1. Llorens J, Navarro J, Martínez Sebastián M, Baylies M, Schneuwly S, Botella J, et al. Causative role of oxidative stress in a Drosophila model of Friedreich ataxia. FASEB J. 2007;21:333-44 pubmed
    ..ataxia (FA), the most common form of hereditary ataxia, is caused by a deficit in the mitochondrial protein frataxin. While several hypotheses have been suggested, frataxin function is not well understood...
  2. Shidara Y, Hollenbeck P. Defects in mitochondrial axonal transport and membrane potential without increased reactive oxygen species production in a Drosophila model of Friedreich ataxia. J Neurosci. 2010;30:11369-78 pubmed publisher
    Friedreich ataxia, a neurodegenerative disorder resulting from frataxin deficiency, is thought to involve progressive cellular damage from oxidative stress...
  3. Anderson P, Kirby K, Hilliker A, Phillips J. RNAi-mediated suppression of the mitochondrial iron chaperone, frataxin, in Drosophila. Hum Mol Genet. 2005;14:3397-405 pubmed
    ..Phenotypically, suppression of the Drosophila frataxin homologue (dfh) confers distinct phenotypes in larvae and adults, leading to giant long-lived larvae and to ..
  4. Runko A, Griswold A, MIN K. Overexpression of frataxin in the mitochondria increases resistance to oxidative stress and extends lifespan in Drosophila. FEBS Lett. 2008;582:715-9 pubmed publisher
    In Friedreich's ataxia, reduction of the mitochondria protein frataxin results in the accumulation of iron and reactive oxygen species, which leads to oxidative damage, neurodegeneration and a diminished lifespan...
  5. Kondapalli K, Kok N, Dancis A, Stemmler T. Drosophila frataxin: an iron chaperone during cellular Fe-S cluster bioassembly. Biochemistry. 2008;47:6917-27 pubmed publisher
    ..In this report, we have characterized the overall stability and iron binding properties of the Drosophila frataxin homologue (Dfh)...
  6. Navarro J, Ohmann E, Sanchez D, Botella J, Liebisch G, Moltó M, et al. Altered lipid metabolism in a Drosophila model of Friedreich's ataxia. Hum Mol Genet. 2010;19:2828-40 pubmed publisher
    ..FRDA) is the most common form of autosomal recessive ataxia caused by a deficit in the mitochondrial protein frataxin. Although demyelination is a common symptom in FRDA patients, no multicellular model has yet been developed to ..
  7. Canizares J, Blanca J, Navarro J, Monros E, Palau F, Molto M. dfh is a Drosophila homolog of the Friedreich's ataxia disease gene. Gene. 2000;256:35-42 pubmed
    ..the Friedreich's ataxia disease gene (FRDA) has been cloned and characterized; it has been named Drosophila frataxin homolog (dfh)...
  8. Soriano S, Calap Quintana P, Llorens J, Al Ramahi I, Gutierrez L, Martínez Sebastián M, et al. Metal Homeostasis Regulators Suppress FRDA Phenotypes in a Drosophila Model of the Disease. PLoS ONE. 2016;11:e0159209 pubmed publisher
    ..inherited ataxia in populations of European origin, is a neurodegenerative disorder caused by a decrease in frataxin levels...
  9. Metzendorf C, Lind M. Drosophila mitoferrin is essential for male fertility: evidence for a role of mitochondrial iron metabolism during spermatogenesis. BMC Dev Biol. 2010;10:68 pubmed publisher
    ..Furthermore, due to the similar expression patterns of some mitochondrial iron metabolism genes in Drosophila and mammals, it is likely that our results are applicable for mammals as well. ..

More Information


  1. Seguin A, Monnier V, Palandri A, Bihel F, Rera M, Schmitt M, et al. A Yeast/Drosophila Screen to Identify New Compounds Overcoming Frataxin Deficiency. Oxid Med Cell Longev. 2015;2015:565140 pubmed publisher
    ..In the present study, we used a yeast strain with a deletion of the frataxin homologue gene as a model of FA cells in a primary screen of two chemical libraries, a fraction of the French National ..
  2. McGurk L, Bonini N. Protein interacting with C kinase (PICK1) is a suppressor of spinocerebellar ataxia 3-associated neurodegeneration in Drosophila. Hum Mol Genet. 2012;21:76-84 pubmed publisher
    ..These findings indicate that interaction proteins may define a rich source of modifier pathways to target in disease situations. ..
  3. Palandri A, L Hôte D, Cohen Tannoudji J, Tricoire H, Monnier V. Frataxin inactivation leads to steroid deficiency in flies and human ovarian cells. Hum Mol Genet. 2015;24:2615-26 pubmed publisher
    ..FA is caused by a GAA repeat expansion in the first intron of the gene encoding frataxin, an evolutionarily conserved mitochondrial protein, which results in decreased gene expression...
  4. Navarro J, Llorens J, Soriano S, Botella J, Schneuwly S, Martínez Sebastián M, et al. Overexpression of human and fly frataxins in Drosophila provokes deleterious effects at biochemical, physiological and developmental levels. PLoS ONE. 2011;6:e21017 pubmed publisher
    ..the most frequent form of inherited ataxias in the Caucasian population, is caused by a reduced expression of frataxin, a highly conserved protein...
  5. Chen K, Lin G, Haelterman N, Ho T, Li T, Li Z, et al. Loss of Frataxin induces iron toxicity, sphingolipid synthesis, and Pdk1/Mef2 activation, leading to neurodegeneration. elife. 2016;5: pubmed publisher
    ..We show that loss of frataxin homolog (fh) in Drosophila leads to iron toxicity, which in turn induces sphingolipid synthesis and ectopically ..
  6. Calap Quintana P, Soriano S, Llorens J, Al Ramahi I, Botas J, Moltó M, et al. TORC1 Inhibition by Rapamycin Promotes Antioxidant Defences in a Drosophila Model of Friedreich's Ataxia. PLoS ONE. 2015;10:e0132376 pubmed publisher
    ..inherited ataxia in the Caucasian population, is a multisystemic disease caused by a significant decrease in the frataxin level...
  7. Rajan A, Perrimon N. Of flies and men: insights on organismal metabolism from fruit flies. BMC Biol. 2013;11:38 pubmed publisher
  8. Soriano S, Llorens J, Blanco Sobero L, Gutierrez L, Calap Quintana P, Morales M, et al. Deferiprone and idebenone rescue frataxin depletion phenotypes in a Drosophila model of Friedreich's ataxia. Gene. 2013;521:274-81 pubmed publisher
    ..inherited ataxia, is a neurodegenerative disease caused by a reduction in the levels of the mitochondrial protein frataxin, the function of which remains a controversial matter...
  9. Wu Z, Du Y, Xue H, Wu Y, Zhou B. Aluminum induces neurodegeneration and its toxicity arises from increased iron accumulation and reactive oxygen species (ROS) production. Neurobiol Aging. 2012;33:199.e1-12 pubmed publisher
    ..Interestingly, Drosophila frataxin (dfh), which causes Friedreich's ataxia if mutated in humans, displayed an interacting effect with Al, suggesting ..
  10. Tricoire H, Palandri A, Bourdais A, Camadro J, Monnier V. Methylene blue rescues heart defects in a Drosophila model of Friedreich's ataxia. Hum Mol Genet. 2014;23:968-79 pubmed publisher
    ..FRDA is caused by abnormally low levels of frataxin, a highly conserved mitochondrial protein...
  11. Navarro J, Botella J, Metzendorf C, Lind M, Schneuwly S. Mitoferrin modulates iron toxicity in a Drosophila model of Friedreich's ataxia. Free Radic Biol Med. 2015;85:71-82 pubmed publisher
    Friedreich's ataxia is the most important recessive ataxia in the Caucasian population. Loss of frataxin expression affects the production of iron-sulfur clusters and, therefore, mitochondrial energy production...
  12. Anderson P, Kirby K, Orr W, Hilliker A, Phillips J. Hydrogen peroxide scavenging rescues frataxin deficiency in a Drosophila model of Friedreich's ataxia. Proc Natl Acad Sci U S A. 2008;105:611-6 pubmed publisher
    ..ataxia (FRDA) is a neurodegenerative disorder arising from a deficit of the mitochondrial iron chaperone, frataxin. Evidence primarily from yeast and mammalian cells is consistent with the hypothesis that a toxic hydroxyl ..
  13. Glasscock E, Singhania A, Tanouye M. The mei-P26 gene encodes a RING finger B-box coiled-coil-NHL protein that regulates seizure susceptibility in Drosophilia. Genetics. 2005;170:1677-89 pubmed
    ..These results reveal a surprising role for mei-P26 outside of the germline as a regulator of seizure susceptibility, possibly by affecting synaptic development as a ubiquitin ligase. ..