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

NEUROBIOLOGY OF DISEASE

卷:48

Article

Peters, Owen M.¹  Millership, Steven¹  Shelkovnikova, Tatyana A.^1,2  Soto, Ileana^3,4  Keeling, Lora¹  Hann, Anthony¹  Marsh-Armstrong, Nicholas^3,4  Buchman, Vladimir L.¹  Ninkina, Natalia^1,2 

[1] Cardiff Univ, Sch Biosci, Cardiff CF10 3AX, S Glam, Wales

[2] Russian Acad Sci, Inst Physiologically Act Cpds RAS, Chernogolovka 142432, Russia

[3] Johns Hopkins Univ, Sch Med, Solomon H Snyder Dept Neurosci, Baltimore, MD USA

[4] Hugo W Moser Res Inst Kennedy Krieger, Baltimore, MD 21205 USA

关键词: Neurodegeneration;    ALS;    Neurofilament;    Aggregation;    Motor neuron;   

DOI  :  10.1016/j.nbd.2012.06.016

来源: Elsevier

【 摘 要 】

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 gamma-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. (C) 2012 Elsevier Inc. All rights reserved.

【 授权许可】

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