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Hokkaido University Collection of Scholarly and Academic Papers >
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Cerebrospinal fluid biomarkers showing neurodegeneration in dogs with GM1 gangliosidosis : possible use for assessment of a therapeutic regimen
Title: | Cerebrospinal fluid biomarkers showing neurodegeneration in dogs with GM1 gangliosidosis : possible use for assessment of a therapeutic regimen |
Authors: | Satoh, Hiroyuki Browse this author | Yamato, Osamu Browse this author | Asano, Tomoya Browse this author | Yonemura, Madoka Browse this author | Yamauchi, Toyofumi Browse this author | Hasegawa, Daisuke Browse this author | Orima, Hiromitsu Browse this author | Arai, Toshiro Browse this author →KAKEN DB | Yamasaki, Masahiro Browse this author →KAKEN DB | Maede, Yoshimitsu Browse this author →KAKEN DB |
Keywords: | Aspartate aminotransferase | Canine model | Cerebrospinal fluid biomarker | Glucocorticoid therapy | GM1 gangliosidosis | Lactate dehydrogenase |
Issue Date: | 16-Feb-2007 |
Publisher: | Elsevier |
Journal Title: | Brain Research |
Volume: | 1133 |
Issue: | 1 |
Start Page: | 200 |
End Page: | 208 |
Publisher DOI: | 10.1016/j.brainres.2006.11.039 |
PMID: | 17196562 |
Abstract: | 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. |
Relation: | http://www.sciencedirect.com/science/journal/00068993 |
Type: | article (author version) |
URI: | http://hdl.handle.net/2115/20150 |
Appears in Collections: | 獣医学院・獣医学研究院 (Graduate School of Veterinary Medicine / Faculty of Veterinary Medicine) > 雑誌発表論文等 (Peer-reviewed Journal Articles, etc)
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Submitter: 前出 吉光
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