ALTERED PURKINJE CELL MIRNA EXPRESSION AND SCA1 PATHOGENESIS

Altered Purkinje cell miRNA expression and SCA1 pathogenesis

Altered Purkinje cell miRNA expression and SCA1 pathogenesis

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Spinocerebellar ataxia type 1 (SCA1) is a dominantly inherited neurodegenerative disorder caused by polyglutamine repeat expansions in Ataxin-1.Recent evidence supports a role for microRNAs (miRNAs) deregulation in SCA1 pathogenesis.However, the extent to which miRNAs may modulate the onset, progression or severity of SCA1 remains largely unknown.In this study, we used here a mouse model of SCA1 to determine if miRNAs are misregulated in pre- and post-symptomatic SCA1 cerebellum.

We found a significant alteration in the steady-state levels of numerous miRNAs prior to and following phenotypic onset.In addition, napoleon concealer we provide evidence that increased miR-150 levels in SCA1 Purkinje neurons may modulate disease pathogenesis by targeting the expression of Rgs8 and Vegfa.

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