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Article

Deranged calcium signaling and neurodegeneration in spinocerebellar ataxia type 3.

The Journal of neuroscience : the official journal of the Society for Neuroscience - 26 Nov 2008

Chen Xi, Tang Tie-Shan, Tu Huiping, Nelson Omar, Pook Mark, Hammer Robert, Nukina Nobuyuki, Bezprozvanny Ilya

Abstract excerpt

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 (ATX3; MJD1) protein. In biochemical experiments, we demonstrate that mutant ATX3(exp) specifically associated with the type 1 inositol 1,4,5-trisphosphate receptor (InsP(3)R1), an intracellular calcium (Ca(2+)) release channel. In...

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Identifiers

PMID: 19036964
DOI: 10.1523/JNEUROSCI.3909-08.2008
PMCID: PMC2663415

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Source receipt: pubmed, retrieved 7 Sept 2026.

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