Abstract
This chapter focuses on inositol 1,4,5-tripshosphate receptor (IP 3 R1), calcium signaling, and polyglutamine expansion disorders. The chapter illustrates that the mutant Huntingtin, ataxin-2, and ataxin-3 proteins specifically bind to the carboxy-terminal region of the type 1 IP 3 R1, an intracellular Ca 2+ release channel. Their association with IP 3 R1 causes sensitization of IP 3 R1to activation by IP 3 in planar lipid bilayers and in neuronal cells. These results suggested that deranged neuronal Ca 2+ signaling might play an important role in pathogenesis of Huntington's disease (HD), spinocerebellar ataxia type 2 (SCA2), and type 3 (SCA3). The chapter demonstrates a connection between abnormal Ca 2+ signaling and neuronal cell death in experiments with HD, SCA2, and SCA3 transgenic mouse models. Based on these results, it is proposed that IP 3 R and other Ca 2+ signaling proteins should be considered as potential therapeutic targets for treatment of HD, SCA2, and SCA3.
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