title: STIM1 Protein Activates Store-Operated Calcium Channels in Cellular Model of Huntington's Disease.
abstract: We have shown that the expression of full-length mutated huntingtin in human neuroblastoma cells (SK-N-SH) leads to an abnormal increase in calcium entry through store-operated channels. In this paper, the expression of the N-terminal fragment of mutated huntingtin (Htt138Q-1exon) is shown to be enough to provide an actual model for Huntington's disease. We have shown that Htt138Q-1exon expression causes increased store-operated calcium entry, which is mediated by at least two types of channels in SK-N-SH cells with different reversal potentials. Calcium sensor, STIM1, is required for activation of store-operated calcium entry in these cells. The results provide grounds for considering the proteins responsible for the activation and maintenance of the store-operated calcium entry as promising targets for developing novel therapeutics for neurodegenerative diseases.
authors.affiliation: Institute of Cytology, Russian Academy of Sciences, Tikhoretsky pr., 4, St. Petersburg, 194064, Russia.
authors.forename: V A
authors.initials: VA
authors.lastname: Vigont
authors.affiliation: Institute of Cytology, Russian Academy of Sciences, Tikhoretsky pr., 4, St. Petersburg, 194064, Russia.
authors.forename: O A
authors.initials: OA
authors.lastname: Zimina
authors.affiliation: Institute of Cytology, Russian Academy of Sciences, Tikhoretsky pr., 4, St. Petersburg, 194064, Russia.
authors.forename: L N
authors.initials: LN
authors.lastname: Glushankova
authors.affiliation: Institute of Cytology, Russian Academy of Sciences, Tikhoretsky pr., 4, St. Petersburg, 194064, Russia.
authors.forename: J A
authors.initials: JA
authors.lastname: Kolobkova
authors.affiliation: Institute of Cytology, Russian Academy of Sciences, Tikhoretsky pr., 4, St. Petersburg, 194064, Russia.
authors.forename: M A
authors.initials: MA
authors.lastname: Ryazantseva
authors.affiliation: Institute of Cytology, Russian Academy of Sciences, Tikhoretsky pr., 4, St. Petersburg, 194064, Russia.
authors.forename: G N
authors.initials: GN
authors.lastname: Mozhayeva
authors.affiliation: Institute of Cytology, Russian Academy of Sciences, Tikhoretsky pr., 4, St. Petersburg, 194064, Russia.
authors.forename: E V
authors.initials: EV
authors.lastname: Kaznacheyeva
keywords: Huntington’s disease; SOC; STIM1; calcium; neurodegeneration