Store-operated calcium entry into SK-N-SH human neuroblastoma cells modeling huntington’s disease

  • V. A. Vigont
  • O. A. Zimina
  • L. N. Glushankova
  • I. B. Bezprozvanny
  • G. N. Mozhayeva
  • E. V. Kaznacheyeva


Huntington’s disease (HD) is an autosomal dominant neurodegenerative disorder caused by expansion of polyglutamine at the N-terminus of the huntingtin protein. Striatal medium spiny neurons (MSN) are the primary targets of HD pathology. In our study, a cellular model of HD was based on the human neuroblastoma cells SK-N-SH transfected with plasmid for expression of the mutant huntingtin protein Htt138Q. Expression of Htt138Q increased store-dependent calcium entry into SK-N-SH cells. EVP4593 reversibly blocked the abnormal store-dependent response, probably generated by the channels incorporating TRPC1 ( transient receptor potential canonical 1) subunit.


Huntington’s disease neurodegeneration calcium SOC TRPC1 


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Copyright information

© Pleiades Publishing, Ltd. 2012

Authors and Affiliations

  • V. A. Vigont
    • 1
  • O. A. Zimina
    • 1
  • L. N. Glushankova
    • 1
  • I. B. Bezprozvanny
    • 2
  • G. N. Mozhayeva
    • 1
  • E. V. Kaznacheyeva
    • 1
  1. 1.Institute of CytologyRussian Academy of SciencesSt. PetersburgRussia
  2. 2.Department of PhysiologyUT Southwestern Medical Center at DallasDallasUSA

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