Friday, July 17, 2015

New fly models of Spinocerebellar ataxia type 17

Hsu TC, Wang CK, Yang CY, Lee LC, Hsieh-Li HM, Ro LS, Chen CM, Lee-Chen GJ, Su MT. Deactivation of TBP contributes to SCA17 pathogenesis. Hum Mol Genet. 2014 Dec 20;23(25):6878-93. PMID: 25104854.

From the abstract: "Spinocerebellar ataxia type 17 (SCA17) is an autosomal dominant cerebellar ataxia caused by the expansion of polyglutamine (polyQ) within the TATA box-binding protein (TBP). ... In this study, we generated novel Drosophila models for SCA17 that recapitulate pathological features such as aggregate formation, mobility defects and premature death. ... downregulation of TBP activity enhances retinal degeneration in SCA3 and Huntington's disease fly models, indicating that the deactivation of TBP is likely to play a common role in polyQ-induced neurodegeneration. "

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