STIM1 Protein Activates Store-Operated Calcium Channels in Cellular Model of Huntington's Disease

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 eno...

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Bibliographic Details
Published in:Actanaturae 2014-10, Vol.6 (4), p.40-47
Main Authors: Vigont, V A, Zimina, O A, Glushankova, L N, Kolobkova, J A, Ryazantseva, M A, Mozhayeva, G N, Kaznacheyeva, E V
Format: Article
Language:English
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Summary: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.
ISSN:2075-8251
DOI:10.32607/20758251-2014-6-4-40-47