scn1lab

Summary

Gene Symbol: scn1lab
Description: sodium channel, voltage-gated, type I like, alpha b
Alias: sodium channel protein type 2 subunit alpha, did, didy, zscn2
Species: zebrafish

Top Publications

  1. Chopra S, Stroud D, Watanabe H, Bennett J, Burns C, Wells K, et al. Voltage-gated sodium channels are required for heart development in zebrafish. Circ Res. 2010;106:1342-50 pubmed publisher
    ..These early defects did not appear to result from altered membrane electrophysiology, as prolonged pharmacological blockade of sodium ..
  2. Novak A, Taylor A, Pineda R, Lasda E, Wright M, Ribera A. Embryonic and larval expression of zebrafish voltage-gated sodium channel alpha-subunit genes. Dev Dyn. 2006;235:1962-73 pubmed
    ..Overall, our findings indicate that scna gene expression occurs sufficiently early during embryogenesis to play developmental roles for both muscle and nervous tissues. ..
  3. Zhang Y, Kecskés A, Copmans D, Langlois M, Crawford A, Ceulemans B, et al. Pharmacological characterization of an antisense knockdown zebrafish model of Dravet syndrome: inhibition of epileptic seizures by the serotonin agonist fenfluramine. PLoS ONE. 2015;10:e0125898 pubmed publisher
    ..We developed a zebrafish model of DS using morpholino antisense oligomers (MOs) targeting scn1Lab, the zebrafish ortholog of SCN1A...
  4. May M, Hwang K, MILES J, Williams C, Niranjan T, Kahler S, et al. ZC4H2, an XLID gene, is required for the generation of a specific subset of CNS interneurons. Hum Mol Genet. 2015;24:4848-61 pubmed publisher
    ..Our findings clearly support ZC4H2 as a novel XLID gene with a required function in interneuron development. Loss of function of ZC4H2 thus likely results in altered connectivity of many brain and spinal circuits. ..
  5. Hong S, Lee P, Baraban S, Lee L. A Novel Long-term, Multi-Channel and Non-invasive Electrophysiology Platform for Zebrafish. Sci Rep. 2016;6:28248 pubmed publisher
    ..Second, we show long-term monitoring during epileptogenic progression in a scn1lab mutant recapitulating human Dravet syndrome...
  6. Novak A, Jost M, Lu Y, Taylor A, Zakon H, Ribera A. Gene duplications and evolution of vertebrate voltage-gated sodium channels. J Mol Evol. 2006;63:208-21 pubmed publisher
    ..Interestingly, despite their different evolutionary histories, mRNA analyses demonstrated a conservation of expression patterns for SCNA orthologues in teleosts and tetrapods, suggesting functional conservation...
  7. Schoonheim P, Arrenberg A, Del Bene F, Baier H. Optogenetic localization and genetic perturbation of saccade-generating neurons in zebrafish. J Neurosci. 2010;30:7111-20 pubmed publisher
    ..analysis, and electrophysiology revealed that the didy mutation disrupts the voltage-gated sodium channel Scn1lab (Nav1.lb)...
  8. Won Y, Ono F, Ikeda S. Characterization of Na+ and Ca2+ channels in zebrafish dorsal root ganglion neurons. PLoS ONE. 2012;7:e42602 pubmed publisher
    ..These findings provide the first comparison of zebrafish and rodent DRG neuron electrical properties and thus provide a basis for future studies. ..
  9. Kumar M, Rowley S, Fulton R, Dinday M, Baraban S, Patel M. Altered Glycolysis and Mitochondrial Respiration in a Zebrafish Model of Dravet Syndrome. Eneuro. 2016;3: pubmed publisher
    ..b>Scn1Lab mutant zebrafish showed a decrease in baseline glycolytic rate and oxygen consumption rate (OCR) compared to ..

More Information

Publications10

  1. Griffin A, Hamling K, Knupp K, Hong S, Lee L, Baraban S. Clemizole and modulators of serotonin signalling suppress seizures in Dravet syndrome. Brain. 2017;140:669-683 pubmed publisher
    ..Our findings demonstrate a rapid path from preclinical discovery in zebrafish, through target identification, to potential clinical treatments for Dravet syndrome. ..