Cerebrospinal fluid biomarkers showing neurodegeneration in dogs with GM1 gangliosidosis : possible use for assessment of a therapeutic regimen

Satoh, Hiroyuki; Yamato, Osamu; Asano, Tomoya; Yonemura, Madoka; Yamauchi, Toyofumi; Hasegawa, Daisuke; Orima, Hiromitsu; Arai, Toshiro; Yamasaki, Masahiro; Maede, Yoshimitsu

Cerebrospinal fluid biomarkers showing neurodegeneration in dogs with GM1 gangliosidosis : possible use for assessment of a therapeutic regimen

Satoh, Hiroyuki; Yamato, Osamu; Asano, Tomoya et al.

Brain Research, 2007, 1133(1), 200-208

Permalink : https://hdl.handle.net/2115/20150

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アクセス権	open access
URI	https://hdl.handle.net/2115/20150
タイトル	Cerebrospinal fluid biomarkers showing neurodegeneration in dogs with GM1 gangliosidosis : possible use for assessment of a therapeutic regimen
著者	Satoh, Hiroyuki ; Yamato, Osamu ; Asano, Tomoya ; Yonemura, Madoka ; Yamauchi, Toyofumi ; Hasegawa, Daisuke ; Orima, Hiromitsu ; Arai, Toshiro ; Yamasaki, Masahiro ; Maede, Yoshimitsu
著者名 Satoh, Hiroyuki
著者名 Yamato, Osamu
著者名 Asano, Tomoya
著者名 Yonemura, Madoka
著者名 Yamauchi, Toyofumi
著者名 Hasegawa, Daisuke
著者名 Orima, Hiromitsu
e-Rad_Researcher 70184257 著者名 Arai, Toshiro
e-Rad_Researcher 40322846 著者名 Yamasaki, Masahiro
e-Rad_Researcher 40002084 著者名 Maede, Yoshimitsu
言語	英語
キーワード	Aspartate aminotransferase
	Canine model
	Cerebrospinal fluid biomarker
	Glucocorticoid therapy
	GM1 gangliosidosis
	Lactate dehydrogenase
発行日	2007-02-16
出版者	Elsevier
収録物名	Brain Research
巻(号)	1133(1)
ページ	200-208
抄録	The present study investigated cerebrospinal fluid (CSF) biomarkers for estimating degeneration of the central nervous system (CNS) in experimental dogs with GM1 gangliosidosis and preliminarily evaluated the efficacy of long-term glucocorticoid therapy for GM1 gangliosidosis using the biomarkers identified here. GM1 gangliosidosis, a lysosomal storage disease that affects the brain and multiple systemic organs, is due to an autosomal recessively inherited deficiency of acid β-galactosidase activity. Pathogenesis of GM1 gangliosidosis may include neuronal apoptosis and abnormal axoplasmic transport and inflammatory response, which are perhaps consequent to massive neuronal storage of GM1 ganglioside. In the present study, we assessed some possible CSF biomarkers, such as GM1 ganglioside, aspartate aminotransferase (AST), lactate dehydrogenase (LDH), neuron-specific enolase (NSE) and myelin basic protein (MBP). Periodic studies demonstrated that GM1 ganglioside concentration, activities of AST and LDH, and concentrations of NSE and MBP in CSF were significantly higher in dogs with GM1 gangliosidosis than those in control dogs, and their changes were well related with the months of age and clinical course. In conclusion, GM1 ganglioside, AST, LDH, NSE and MBP could be utilized as CSF biomarkers showing CNS degeneration in dogs with GM1 gangliosidosis to evaluate the efficacy of novel therapies proposed for this disease. In addition, we preliminarily treated an affected dog with long-term oral administration of prednisolone and evaluated the efficacy of this therapeutic trial using CSF biomarkers determined in the present study. However, this treatment did not change either the clinical course or the CSF biomarkers of the affected dog, suggesting that glucocorticoid therapy would not be effective for treating GM1 gangliosidosis.
資源タイプ	学術雑誌論文
出版タイプ	Accepted Manuscript
PISSN	0006-8993
EISSN	0006-8993
関連情報	PMID 17196562
関連情報	URI http://www.sciencedirect.com/science/journal/00068993
関連情報 (isVersionOf)	出版社版DOI https://doi.org/10.1016/j.brainres.2006.11.039