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

C. J. L. M. Smeets, D. S. Verbeek*

*Corresponding author for this work

Research output: Contribution to journalReview articleAcademicpeer-review

24 Citations (Scopus)

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. (C) 2016 Elsevier Inc. All rights reserved.

Original languageEnglish
Pages (from-to)96-106
Number of pages11
JournalNeurobiology of Disease
Volume88
DOIs
Publication statusPublished - Apr-2016

Keywords

  • Spinocerebellar ataxia
  • Climbing fibers
  • Synaptic plasticity
  • Glutamate
  • Common pathology
  • Mouse models
  • LONG-TERM DEPRESSION
  • CEREBELLAR PURKINJE-CELLS
  • BETA-III SPECTRIN
  • CAG TRINUCLEOTIDE REPEAT
  • M-AAA PROTEASE
  • MUTATIONS CAUSE
  • SYNAPTIC PLASTICITY
  • CALCIUM TRANSIENTS
  • NMDA RECEPTOR
  • COMPLEX SPIKE

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