Climbing fibers in spinocerebellar ataxia: A mechanism for the loss of motor control

C J L M Smeets; D S Verbeek

doi:10.1016/j.nbd.2016.01.009

Climbing fibers in spinocerebellar ataxia: A mechanism for the loss of motor control

Neurobiol Dis. 2016 Apr:88:96-106. doi: 10.1016/j.nbd.2016.01.009. Epub 2016 Jan 12.

Authors

C J L M Smeets¹, D S Verbeek²

Affiliations

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

PMID: 26792399
DOI: 10.1016/j.nbd.2016.01.009

Abstract

The spinocerebellar ataxias (SCAs) form an ever-growing group of neurodegenerative disorders causing dysfunction of the cerebellum and loss of motor control in patients. Currently, 41 different genetic causes have been identified, with each mutation affecting a different gene. Interestingly, these diverse genetic causes all disrupt cerebellar function and produce similar symptoms in patients. In order to understand the disease better, and define possible therapeutic targets for multiple SCAs, the field has been searching for common ground among the SCAs. In this review, we discuss the physiology of climbing fibers and the possibility that climbing fiber dysfunction is a point of convergence for at least a subset of SCAs.

Keywords: Climbing fibers; Common pathology; Glutamate; Mouse models; Spinocerebellar ataxia; Synaptic plasticity.

Publication types

Research Support, Non-U.S. Gov't
Review

MeSH terms

Animals
Cerebellum / pathology*
Humans
Movement Disorders / etiology*
Nerve Fibers / metabolism*
Spinocerebellar Ataxias / complications*
Spinocerebellar Ataxias / genetics
Spinocerebellar Ataxias / pathology*