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 journalArticle

14 Citations (Scopus)

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

Fingerprint

gamma-Synuclein
Amyotrophic Lateral Sclerosis
Motor Neurons
Transgenic Mice
Synucleins
Pathology
Cognition
Axons
Population
Proteins

Keywords

  • Aggregation
  • ALS
  • Motor neuron
  • Neurodegeneration
  • Neurofilament

ASJC Scopus subject areas

  • Neurology

Cite this

Selective pattern of motor system damage in gamma-synuclein transgenic mice mirrors the respective pathology in amyotrophic lateral sclerosis. / Peters, Owen M.; Millership, Steven; Shelkovnikova, Tatyana A.; Soto, Ileana; Keeling, Lora; Hann, Anthony; Marsh-Armstrong, Nicholas; Buchman, Vladimir L.; Ninkina, Natalia.

In: Neurobiology of Disease, Vol. 48, No. 1, 01.10.2012, p. 124-131.

Research output: Contribution to journalArticle

Peters, Owen M. ; Millership, Steven ; Shelkovnikova, Tatyana A. ; Soto, Ileana ; Keeling, Lora ; Hann, Anthony ; Marsh-Armstrong, Nicholas ; Buchman, Vladimir L. ; Ninkina, Natalia. / Selective pattern of motor system damage in gamma-synuclein transgenic mice mirrors the respective pathology in amyotrophic lateral sclerosis. In: Neurobiology of Disease. 2012 ; Vol. 48, No. 1. pp. 124-131.
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