Localization of a mutant SOD1 protein in E40K-heterozygous dogs: Implications for non-cell-autonomous pathogenesis of degenerative myelopathy

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• 作者：Yui Kobatake^a ; Hiroki Sakai^a ; ^b ; Toshihiro Tsukui^c ; Osamu Yamato^d ; Moeko Kohyama^d ; Jun Sasaki^e ; Shinsuke Kato^f ; Makoto Urushitani^g ; Sadatoshi Maeda^a ; ^b ; Hiroaki Kamishina^a ; ^b ; ^h ; ^{kamicna@gifu-u.ac.jp}
• 关键词：Canine ; Animal model ; Degenerative myelopathy ; Astrocyte ; Amyotrophic lateral sclerosis ; Superoxide dismutase 1
• 刊名：Journal of the Neurological Sciences
• 出版年：2017
• 出版时间：15 January 2017
• 年：2017
• 卷：372
• 期：Complete
• 页码：369-378
• 全文大小：2666 K
• 卷排序：372

文摘

Canine degenerative myelopathy (DM) is a potential spontaneous model of ALS. Aggregates of mutant SOD1 accumulated in spinal neurons of symptomatic DM. Spinal neurons were free of mutant SOD1 aggregates during the early phase of DM. The mutant SOD1 proteins accumulated in astrocytes during the early phase of DM. The results imply the importance of non-cell autonomous mechanism of DM pathogenesis.