@article{cd9abaaedf694e12bc4f106fda8c4aa6,
title = "Store-operated calcium entry into SK-N-SH human neuroblastoma cells modeling Huntington's disease",
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.",
keywords = "Calcium, Huntington's disease, Neurodegeneration, SOC, TRPC1",
author = "Vigont, {V. A.} and Zimina, {O. A.} and Glushankova, {L. N.} and Bezprozvanny, {I. B.} and Mozhayeva, {G. N.} and Kaznacheyeva, {E. V.}",
note = "Funding Information: ACKNOWLEDGMENTS The authors are grateful to EnVivo Inc. (USA) for a generous gift of EVP compounds; to Dr. L. Tsiokas (University of Oklahoma Health Science Center), for the plasmid encoding anti TRPC1 siRNA, and to Dr. S. Muallem (NIH, USA), for a construct for TRPC1 expression. The work was supported by the Ministry of Science and Education (State contract no. P332, 14.740.11.0924), by the Program of the Presid ium of Russian Academy of Sciences Molecular and Cell Biology, by the Russian Foundation for Basic Research (project nos. 11 04 12047, 10 04 01002, and 10 04 00956), and the Program in Support of the Leading Scientific Schools (project no. SS 3796.2010.4).",
year = "2012",
month = apr,
doi = "10.1134/S199074781201014X",
language = "English (US)",
volume = "6",
pages = "206--214",
journal = "Biochemistry (Moscow) Supplement Series A: Membrane and Cell Biology",
issn = "1990-7478",
publisher = "Maik Nauka-Interperiodica Publishing",
number = "2",
}