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

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.

journal_name

Neurobiol Dis

journal_title

Neurobiology of disease

authors

Peters OM,Millership S,Shelkovnikova TA,Soto I,Keeling L,Hann A,Marsh-Armstrong N,Buchman VL,Ninkina N

doi

10.1016/j.nbd.2012.06.016

subject

Has Abstract

pub_date

2012-10-01 00:00:00

pages

124-31

issue

1

eissn

0969-9961

issn

1095-953X

pii

S0969-9961(12)00231-8

journal_volume

48

pub_type

杂志文章

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