Selective pattern of motor system damage in gamma-synuclein transgenic mice mirrors the respective pathology in amyotrophic lateral sclerosis

Owen M. Peters, Steven Millership, Tatyana A. Shelkovnikova, Ileana Soto, Lora Keeling, Anthony Hann, Nicholas Marsh-Armstrong, Vladimir L. Buchman, Natalia Ninkina

Research output: Contribution to journalArticlepeer-review

20 Scopus citations

Abstract

Amyotrophic lateral sclerosis (ALS) is characterised by substantial loss of both upper and lower motor neuron function, with sensory and cognitive systems less affected. Though heritable forms of the disease have been described, the vast majority of cases are sporadic with poorly defined underlying pathogenic mechanisms. Here we demonstrate that the neurological pathology induced in transgenic mice by overexpression of γ-synuclein, a protein not previously associated with ALS, recapitulates key features of the disease, namely selective damage and loss of discrete populations of upper and lower motor neurons and their axons, contrasted by limited effects upon the sensory system.

Original languageEnglish (US)
Pages (from-to)124-131
Number of pages8
JournalNeurobiology of Disease
Volume48
Issue number1
DOIs
StatePublished - Oct 1 2012
Externally publishedYes

Keywords

  • Aggregation
  • ALS
  • Motor neuron
  • Neurodegeneration
  • Neurofilament

ASJC Scopus subject areas

  • Neurology

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