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Neurobiol Dis. 2012 Oct;48(1):124-31. doi: 10.1016/j.nbd.2012.06.016. Epub 2012 Jun 30.

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

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  • 1School of Biosciences, Cardiff University, Museum Avenue, Cardiff, CF10 3AX, UK.

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.

Copyright © 2012 Elsevier Inc. All rights reserved.

PMID:
22750530
[PubMed - indexed for MEDLINE]
PMCID:
PMC3457776
Free PMC Article
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