Abstract
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.
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Original Russian Text © V.A. Vigont, O.A. Zimina, L.N. Glushankova, I.B. Bezprozvanny, G.N. Mozhayeva, E.V. Kaznacheyeva, 2012, published in Biologicheskie Membrany, 2012, Vol. 29, Nos. 1–2, pp. 123–132.
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Vigont, V.A., Zimina, O.A., Glushankova, L.N. et al. Store-operated calcium entry into SK-N-SH human neuroblastoma cells modeling huntington’s disease. Biochem. Moscow Suppl. Ser. A 6, 206–214 (2012). https://doi.org/10.1134/S199074781201014X
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DOI: https://doi.org/10.1134/S199074781201014X