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

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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.
Type: article (author version)
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)

Submitter: 前出 吉光

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