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

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• 作者：Owen M. Peters ; Steven Millership ; Tatyana A. Shelkovnikova ; Ileana Soto ; Lora Keeling ; Anthony Hann ; Nicholas Marsh-Armstrong ; Vladimir L. Buchman ; Natalia Ninkina
• 关键词：ALS ; Amyotrophic lateral sclerosis ; DRG ; Dorsal root ganglion ; TDP-43 ; TAR DNA binding protein-43 ; FUS/TLS ; Fused in sarcoma/translocated in liposarcoma ; RGC ; Retinal ganglion cell ; NF ; Neurofilament ; pNF ; Phosphorylated neurofilament
• 刊名：Neurobiology of Disease
• 出版年：2012
• 出版时间：October, 2012
• 年：2012
• 卷：48
• 期：1
• 页码：124-131
• 全文大小：1678 K

文摘

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 ¦Ã-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.