Gene Symbol: ND-30
Description: NADH dehydrogenase (ubiquinone) 30 kDa subunit
Alias: C-I 30, C-I-NDUFS3, CG 12079, CG12079, Dmel\CG12079, NDUFS3, NDufS3, NUGM, NADH dehydrogenase (ubiquinone) 30 kDa subunit, 30 kDa subunit of complex-I, CG12079-PA, NADH dehydrogenase (ubiquinone) Fe-S protein 3, NADH ubiquinone oxidoreductase 30 kDa, NADH-UBIQUINONE OXIDOREDUCTASE 30 KDA SUBUNIT, NADH:ubiquinone oxidoreductase 30 kDa subunit, ND-30-PA, nuclear-encoded subunit NDUFS3 of complex I
Species: fruit fly

Top Publications

  1. Hao L, Giasson B, Bonini N. DJ-1 is critical for mitochondrial function and rescues PINK1 loss of function. Proc Natl Acad Sci U S A. 2010;107:9747-52 pubmed publisher
    ..These findings link DJ-1, pink1, and parkin to mitochondrial integrity and provide the foundation for therapeutics that link bioenergetics and parkinsonism. ..
  2. Pimenta de Castro I, Costa A, Lam D, Tufi R, Fedele V, Moisoi N, et al. Genetic analysis of mitochondrial protein misfolding in Drosophila melanogaster. Cell Death Differ. 2012;19:1308-16 pubmed publisher
    ..We show that in flies, a pathway involving pink1, parkin and ref(2)P has a role in the maintenance of a viable pool of cellular mitochondria by promoting organellar quality control. ..
  3. Hur J, Stork D, Walker D. Complex-I-ty in aging. J Bioenerg Biomembr. 2014;46:329-35 pubmed publisher
    ..We suggest that manipulations that increase or decrease complex I activity both converge on improved mitochondrial homeostasis during aging, resulting in prolonged lifespan. ..
  4. Celotto A, Chiu W, Van Voorhies W, Palladino M. Modes of metabolic compensation during mitochondrial disease using the Drosophila model of ATP6 dysfunction. PLoS ONE. 2011;6:e25823 pubmed publisher
    ..These studies demonstrate the dynamic nature of metabolic compensatory mechanisms and emphasize the need for time course studies in tractable animal systems to elucidate disease pathogenesis and novel therapeutic avenues. ..
  5. Vilain S, Esposito G, Haddad D, Schaap O, Dobreva M, Vos M, et al. The yeast complex I equivalent NADH dehydrogenase rescues pink1 mutants. PLoS Genet. 2012;8:e1002456 pubmed publisher
  6. 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
  7. Lee K, Wu Z, Song Y, Mitra S, Feroze A, Cheshier S, et al. Roles of PINK1, mTORC2, and mitochondria in preserving brain tumor-forming stem cells in a noncanonical Notch signaling pathway. Genes Dev. 2013;27:2642-7 pubmed publisher
    ..Our results emphasize the importance of mitochondria to N and NSC biology, with important implications for diseases associated with aberrant N signaling. ..
  8. Shiba Fukushima K, Inoshita T, Hattori N, Imai Y. PINK1-mediated phosphorylation of Parkin boosts Parkin activity in Drosophila. PLoS Genet. 2014;10:e1004391 pubmed publisher
  9. Lee J, Treisman J. Sightless has homology to transmembrane acyltransferases and is required to generate active Hedgehog protein. Curr Biol. 2001;11:1147-52 pubmed
    ..sit encodes a conserved transmembrane protein with homology to a family of membrane-bound acyltransferases. The Sit protein could act by acylating Hh or by promoting other modifications or trafficking events necessary for its function. ..

More Information


  1. Chamoun Z, Mann R, Nellen D, von Kessler D, Bellotto M, Beachy P, et al. Skinny hedgehog, an acyltransferase required for palmitoylation and activity of the hedgehog signal. Science. 2001;293:2080-4 pubmed
  2. 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
    ..Our results provide in vivo evidence of a functional equivalence for human and fly frataxins and indicate that the control of frataxin expression is important for treatments that aim to increase frataxin levels. ..
  3. Thomas R, Andrews L, Burman J, Lin W, Pallanck L. PINK1-Parkin pathway activity is regulated by degradation of PINK1 in the mitochondrial matrix. PLoS Genet. 2014;10:e1004279 pubmed publisher
    ..Our findings thus suggest that Lon plays an essential role in regulating the PINK1-Parkin pathway by promoting the degradation of PINK1 in the matrix of healthy mitochondria. ..
  4. Scialò F, Sriram A, Fernández Ayala D, Gubina N, Lõhmus M, Nelson G, et al. Mitochondrial ROS Produced via Reverse Electron Transport Extend Animal Lifespan. Cell Metab. 2016;23:725-34 pubmed publisher
    ..These results illustrate that the source of a ROS signal is vital in determining its effects on cellular physiology and establish that manipulation of ubiquinone redox state is a valid strategy to delay aging. ..
  5. Sieber M, Thomsen M, Spradling A. Electron Transport Chain Remodeling by GSK3 during Oogenesis Connects Nutrient State to Reproduction. Cell. 2016;164:420-32 pubmed publisher
    ..Our studies reveal an important link between metabolism and oocyte maturation. ..
  6. Sing A, Tsatskis Y, Fabian L, Hester I, Rosenfeld R, Serricchio M, et al. The atypical cadherin fat directly regulates mitochondrial function and metabolic state. Cell. 2014;158:1293-1308 pubmed publisher
    ..Defects in mitochondrial activity in ft mutants are independent of Hippo and PCP signaling and are reminiscent of the Warburg effect. ..
  7. de Castro I, Costa A, Celardo I, Tufi R, Dinsdale D, Loh S, et al. Drosophila ref(2)P is required for the parkin-mediated suppression of mitochondrial dysfunction in pink1 mutants. Cell Death Dis. 2013;4:e873 pubmed publisher
    ..We conclude that Ref(2)P is a crucial downstream effector of a pathway involving Pink1 and Parkin and is responsible for the maintenance of a viable pool of cellular mitochondria by promoting their aggregation and autophagic clearance. ..
  8. Zhang K, Li Z, Jaiswal M, Bayat V, Xiong B, Sandoval H, et al. The C8ORF38 homologue Sicily is a cytosolic chaperone for a mitochondrial complex I subunit. J Cell Biol. 2013;200:807-20 pubmed publisher
    ..Our data indicate that cytosolic chaperones are required for the subcellular transport of ND42. ..
  9. Wredenberg A, Lagouge M, Bratic A, Metodiev M, Spåhr H, Mourier A, et al. MTERF3 regulates mitochondrial ribosome biogenesis in invertebrates and mammals. PLoS Genet. 2013;9:e1003178 pubmed publisher
  10. Liu W, Acin Perez R, Geghman K, Manfredi G, Lu B, Li C. Pink1 regulates the oxidative phosphorylation machinery via mitochondrial fission. Proc Natl Acad Sci U S A. 2011;108:12920-4 pubmed publisher
    ..Taken together, these results suggest a unique pathogenic mechanism of PINK1 PD: The loss of PINK1 impairs mitochondrial fission, which causes defective assembly of the ETC complexes, leading to abnormal bioenergetics. ..
  11. Sen A, Kalvakuri S, Bodmer R, Cox R. Clueless, a protein required for mitochondrial function, interacts with the PINK1-Parkin complex in Drosophila. Dis Model Mech. 2015;8:577-89 pubmed publisher
    ..Taken together, these results suggest that Clu directly modulates mitochondrial function, and that Clu's function contributes to the PINK1-Park pathway of mitochondrial quality control. ..
  12. Shirasaki D, Greiner E, Al Ramahi I, Gray M, Boontheung P, Geschwind D, et al. Network organization of the huntingtin proteomic interactome in mammalian brain. Neuron. 2012;75:41-57 pubmed publisher