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DC Field | Value | Language |
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dc.contributor.author | Chen, X | - |
dc.contributor.author | Tang, TS | - |
dc.contributor.author | Tu, H | - |
dc.contributor.author | Nelson, O | - |
dc.contributor.author | Pook, M | - |
dc.contributor.author | Hammer, R | - |
dc.contributor.author | Nukina, N | - |
dc.contributor.author | Bezprozvanny, I | - |
dc.date.accessioned | 2009-03-20T12:40:49Z | - |
dc.date.available | 2009-03-20T12:40:49Z | - |
dc.date.issued | 2008 | - |
dc.identifier.citation | The Journal of Neuroscience. 28(48): 12713-12724 | en |
dc.identifier.uri | http://bura.brunel.ac.uk/handle/2438/3132 | - |
dc.description.abstract | Spinocerebellar ataxia type 3 (SCA3), also known as Machado-Joseph disease (MJD), is an autosomal-dominant neurodegenerative disorder caused by a polyglutamine expansion in ataxin-3 (SCA3, MJD1) protein. In biochemical experiments we demonstrate that mutant SCA3exp specifically associated with the type 1 inositol 1,4,5-trisphosphate receptor (InsP3R1), an intracellular calcium (Ca2+) release channel. In electrophysiological and Ca2+ imaging experiments we show that InsP3R1 are sensitized to activation by InsP3 in the presence of mutant SCA3exp. We found that feeding SCA3-YAC-84Q transgenic mice with dantrolene, a clinically relevant stabilizer of intracellular Ca2+ signaling, improved their motor performance and prevented neuronal cells loss in pontine nuclei and substantia nigra regions. Our results indicate that deranged Ca2+ signaling may play an important role in SCA3 pathology and that Ca2+ signaling stabilizers such as dantrolene may be considered as potential therapeutic drugs for treatment of SCA3 patients. | en |
dc.format.extent | 565225 bytes | - |
dc.format.mimetype | application/pdf | - |
dc.language.iso | en | - |
dc.publisher | Society for Neuroscience | - |
dc.subject | SCA3 | en |
dc.subject | MJD | en |
dc.title | Deranged calcium signaling and neurodegeneration in spinocerebellar ataxia type 3 | en |
dc.type | Research Paper | en |
dc.identifier.doi | http://dx.doi.org/10.1523/JNEUROSCI.3909-08.2008 | - |
Appears in Collections: | Community Health and Public Health Dept of Life Sciences Research Papers |
Files in This Item:
File | Description | Size | Format | |
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Chen submitted.pdf | 551.98 kB | Adobe PDF | View/Open |
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