Spinocerebellar ataxia: miRNAs expose biological pathways underlying pervasive Purkinje cell degeneration

Rogier van der Stijl; Sebo Withoff; Dineke S Verbeek

doi:10.1016/j.nbd.2017.08.003

Spinocerebellar ataxia: miRNAs expose biological pathways underlying pervasive Purkinje cell degeneration

Neurobiol Dis. 2017 Dec:108:148-158. doi: 10.1016/j.nbd.2017.08.003. Epub 2017 Aug 18.

Authors

Rogier van der Stijl¹, Sebo Withoff¹, Dineke S Verbeek²

Affiliations

¹ Department of Genetics, University of Groningen, University Medical Centre Groningen, Groningen, The Netherlands.
² Department of Genetics, University of Groningen, University Medical Centre Groningen, Groningen, The Netherlands. Electronic address: d.s.verbeek@umcg.nl.

PMID: 28823930
DOI: 10.1016/j.nbd.2017.08.003

Abstract

Recent work has demonstrated the importance of miRNAs in the pathogenesis of various brain disorders including the neurodegenerative disorder spinocerebellar ataxia (SCA). This review focuses on the role of miRNAs in the shared pathogenesis of the different SCA types. We examine the novel findings of a recent cell-type-specific RNA-sequencing study in mouse brain and discuss how the identification of Purkinje-cell-enriched miRNAs highlights biological pathways that expose the mechanisms behind pervasive Purkinje cell degeneration in SCA. These key pathways are likely to contain targets for therapeutic development and represent potential candidate genes for genetically unsolved SCAs.

Keywords: Cerebellum; Neurodegeneration; Purkinje cells; Spinocerebellar ataxia; miRNA.

Publication types

Review

MeSH terms

Animals
Humans
MicroRNAs / metabolism*
Nerve Degeneration / metabolism*
Purkinje Cells / metabolism*
Spinocerebellar Ataxias / metabolism*

Substances

MicroRNAs