Huntington's disease (HD) is an autosomal dominant neurodegenerative disorder caused by expansion of polyglutamine on 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 anomalous store-dependent response, probably generated by the channels incorporating TRPC1 (short transient receptor potential channel 1) subunit.
|Original language||English (US)|
|Number of pages||10|
|State||Published - Dec 3 2012|
ASJC Scopus subject areas
- Molecular Biology
- Cell Biology