Gene Symbol: Surf1
Description: Surfeit 1
Alias: BcDNA:GH23574, CG9943, Dmel\CG9943, SUR1, surfeit 1, CG9943-PA, Surf1-PA, surfeit locus protein 1
Species: fruit fly

Top Publications

  1. Fernandez Ayala D, Sanz A, Vartiainen S, Kemppainen K, Babusiak M, Mustalahti E, et al. Expression of the Ciona intestinalis alternative oxidase (AOX) in Drosophila complements defects in mitochondrial oxidative phosphorylation. Cell Metab. 2009;9:449-60 pubmed publisher complement the semilethality of partial knockdown of both cyclope (COXVIc) and the complex IV assembly factor Surf1. It also rescued the locomotor defect and excess mitochondrial ROS production of flies mutated in dj-1beta, a ..
  2. Poyau A, Buchet K, Godinot C. Sequence conservation from human to prokaryotes of Surf1, a protein involved in cytochrome c oxidase assembly, deficient in Leigh syndrome. FEBS Lett. 1999;462:416-20 pubmed
    The human SURF1 gene encoding a protein involved in cytochrome c oxidase (COX) assembly, is mutated in most patients presenting Leigh syndrome associated with COX deficiency...
  3. Zordan M, Cisotto P, Benna C, Agostino A, Rizzo G, Piccin A, et al. Post-transcriptional silencing and functional characterization of the Drosophila melanogaster homolog of human Surf1. Genetics. 2006;172:229-41 pubmed
    Mutations in Surf1, a human gene involved in the assembly of cytochrome c oxidase (COX), cause Leigh syndrome, the most common infantile mitochondrial encephalopathy, characterized by a specific COX deficiency...
  4. Sotillos S, Espinosa Vázquez J, Foglia F, Hu N, Hombria J. An efficient approach to isolate STAT regulated enhancers uncovers STAT92E fundamental role in Drosophila tracheal development. Dev Biol. 2010;340:571-82 pubmed publisher
    ..Our results indicate that vvl and trh are independently activated by STAT92E which is the most important transcription factor required for trachea specification. ..
  5. Cagin U, Duncan O, Gatt A, Dionne M, Sweeney S, Bateman J. Mitochondrial retrograde signaling regulates neuronal function. Proc Natl Acad Sci U S A. 2015;112:E6000-9 pubmed publisher
    ..Thus, mitochondrial retrograde signaling regulates neuronal activity and can be manipulated to enhance neuronal function, despite mitochondrial impairment. ..
  6. Kemppainen K, Rinne J, Sriram A, Lakanmaa M, Zeb A, Tuomela T, et al. Expression of alternative oxidase in Drosophila ameliorates diverse phenotypes due to cytochrome oxidase deficiency. Hum Mol Genet. 2014;23:2078-93 pubmed publisher
    ..These findings confirm the potential utility of alternative respiratory chain enzymes as tools to combat mitochondrial disease, while indicating important limitations thereof. ..
  7. Da Rè C, von Stockum S, Biscontin A, Millino C, Cisotto P, Zordan M, et al. Leigh syndrome in Drosophila melanogaster: morphological and biochemical characterization of Surf1 post-transcriptional silencing. J Biol Chem. 2014;289:29235-46 pubmed publisher
    ..We established a Drosophila melanogaster model of LS based on the post-transcriptional silencing of CG9943, the Drosophila homolog of SURF1...
  8. Martínez Morentin L, Martínez L, Piloto S, Yang H, Schon E, Garesse R, et al. Cardiac deficiency of single cytochrome oxidase assembly factor scox induces p53-dependent apoptosis in a Drosophila cardiomyopathy model. Hum Mol Genet. 2015;24:3608-22 pubmed publisher
    ..Remarkably, apoptosis is enhanced in the muscle and liver of Sco2 knock-out mice, clearly suggesting that cell death is a key feature of the COX deficiencies produced by mutations in Sco genes in humans. ..