Format

Send to

Choose Destination
Neurobiol Dis. 2013 Jun;54:456-63. doi: 10.1016/j.nbd.2013.01.019. Epub 2013 Jan 30.

Altered Purkinje cell miRNA expression and SCA1 pathogenesis.

Author information

1
Department of Internal Medicine, University of Iowa, Iowa City, IA 52242, USA.

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.

PMID:
23376683
PMCID:
PMC3629010
DOI:
10.1016/j.nbd.2013.01.019
[Indexed for MEDLINE]
Free PMC Article

Supplemental Content

Full text links

Icon for Elsevier Science Icon for PubMed Central
Loading ...
Support Center