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Publication Detail

Title: Altered Purkinje cell miRNA expression and SCA1 pathogenesis.

Authors: Rodriguez-Lebron, Edgardo; Liu, Gumei; Keiser, Megan; Behlke, Mark A; Davidson, Beverly L

Published In Neurobiol Dis, (2013 Jun)

Abstract: 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 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, we provide evidence that increased miR-150 levels in SCA1 Purkinje neurons may modulate disease pathogenesis by targeting the expression of Rgs8 and Vegfa.

PubMed ID: 23376683 Exiting the NIEHS site

MeSH Terms: No MeSH terms associated with this publication

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