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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Detalles Bibliográficos
Autores principales: Vigont, V. A., Zimina, O. A., Glushankova, L. N., Kolobkova, J. A., Ryazantseva, M. A., Mozhayeva, G. N., Kaznacheyeva, E. V.
Formato: Online Artículo Texto
Lenguaje:English
Publicado: A.I. Gordeyev 2014
Materias:
Research Article
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4273090/
https://www.ncbi.nlm.nih.gov/pubmed/25558393
Descripción
Sumario: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.

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