In vitro evidence of propagation of superoxide dismutase-1 protein aggregation in canine degenerative myelopathy

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In vitro evidence of propagation of superoxide dismutase-1 protein aggregation in canine degenerative myelopathy

https://gifu-pu.repo.nii.ac.jp/records/14558

Item type		研究室原著論文(1)
公開日		2022-03-07
タイトル
	タイトル	In vitro evidence of propagation of superoxide dismutase-1 protein aggregation in canine degenerative myelopathy
タイトル
	タイトル	In vitro evidence of propagation of superoxide dismutase-1 protein aggregation in canine degenerative myelopathy
	言語	en
言語
	言語	eng
資源タイプ
	資源タイプ識別子	http://purl.org/coar/resource_type/c_6501
	資源タイプ	journal article
アクセス権
	アクセス権	metadata only access
	アクセス権URI	http://purl.org/coar/access_right/c_14cb
抄録
	値	Canine degenerative myelopathy (DM) is a progressive and fatal neurodegenerative disorder that has been linked to mutations in the superoxide dismutase 1 (SOD1) gene. The accumulation of misfolded protein aggregates in spinal neurons and astrocytes is implicated as an important pathological process in DM; however, the mechanism of protein aggregate formation is largely unknown. In human neurodegenerative diseases, such as amyotrophic lateral sclerosis (ALS), cell-to-cell propagation of disease-relevant proteins has been demonstrated. Therefore, in this study, propagation of aggregation-forming property of mutant SOD1 protein in DM in vitro was investigated. This study demonstrated that aggregates composed of canine wild type SOD1 protein were increased by co-transfection with canine mutant SOD1 (E40K SOD1), indicating intracellular propagation of SOD1 aggregates. Further, aggregated recombinant SOD1 proteins were released from the cells, taken up by other cells, and induced further aggregate formation of normally folded SOD1 proteins. These results suggest intercellular propagation of SOD1 aggregates. The hypothesis of cell-to-cell propagation of SOD1 aggregates proposed in this study may underly the progressive nature of DM pathology.
書誌情報		en : The Veterinary Journal 巻 274, p. 105710, 発行日 2021