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

Peters, Owen Morgan, Millership, Steven, Shelkovnikova, Tatyana, Soto, Ileana, Keeling, Lora, Hann, Anthona, Marsh-Armstrong, Nicholas, Buchman, Vladimir L. and Ninkina, Natalia 2012. Selective pattern of motor system damage in gamma-synuclein transgenic mice mirrors the respective pathology in amyotrophic lateral sclerosis. Neurobiology of Disease 48 (1) , pp. 124-131. 10.1016/j.nbd.2012.06.016

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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.

Item Type: Article
Date Type: Publication
Status: Published
Schools: Biosciences
Subjects: R Medicine > RC Internal medicine > RC0321 Neuroscience. Biological psychiatry. Neuropsychiatry
Uncontrolled Keywords: Neurodegeneration; ALS; Neurofilament; Aggregation; Motor neuron
Publisher: Elsevier
ISSN: 0969-9961
Last Modified: 11 Sep 2019 21:16
URI: http://orca.cf.ac.uk/id/eprint/41843

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