Art

J-GLOBAL ID：201702233078266970   Reference number：17A0358837

Efficacy of bezafibrate on fibroblasts of glutaric acidemia type II patients evaluated using an in vitro probe acylcarnitine assay

in vitroプローブアシルカルニチンアッセイを用いて評価したグルタル酸血症II型患者線維芽細胞に対するベザフィブラートの効果

Author (11)： Yamada Kenji (Department of Pediatrics, Shimane University, Faculty of Medicine, Izumo, Shimane, Japan) ,  Kobayashi Hironori (Department of Pediatrics, Shimane University, Faculty of Medicine, Izumo, Shimane, Japan) ,  Bo Ryosuke (Department of Pediatrics, Shimane University, Faculty of Medicine, Izumo, Shimane, Japan) ,  Bo Ryosuke (Department of Pediatrics, Kobe University Graduate School of Medicine, Kobe, Hyogo, Japan) ,  Purevsuren Jamiyan (Department of Pediatrics, Shimane University, Faculty of Medicine, Izumo, Shimane, Japan) ,  Mushimoto Yuichi (Department of Pediatrics, Shimane University, Faculty of Medicine, Izumo, Shimane, Japan) ,  Takahashi Tomoo (Department of Pediatrics, Shimane University, Faculty of Medicine, Izumo, Shimane, Japan) ,  Hasegawa Yuki (Department of Pediatrics, Shimane University, Faculty of Medicine, Izumo, Shimane, Japan) ,  Taketani Takeshi (Department of Pediatrics, Shimane University, Faculty of Medicine, Izumo, Shimane, Japan) ,  Fukuda Seiji (Department of Pediatrics, Shimane University, Faculty of Medicine, Izumo, Shimane, Japan) ,  Yamaguchi Seiji (Department of Pediatrics, Shimane University, Faculty of Medicine, Izumo, Shimane, Japan)
Material： Brain & Development  (Brain & Dev)
Volume： 39  Issue： 1  Page： 48-57  Publication year： Jan. 2017 
JST Material Number： W0814A  ISSN： 0387-7604  Document type： Article
Article type： 原著論文  Country of issue： Netherlands (NLD)  Language： ENGLISH (EN)

Thesaurus term： human ,...
Semi thesaurus term： *acylcarnitine ,...

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Author keywords (5)： Bezafibrate ,  Glutaric acidemia type II ,  Multiple acyl-CoA dehydrogenase deficiency ,  In vitro probe assay ,  Fatty acid oxidation disorders

JST classification (3)： Basic research of drugs acting on lipid metabolism  (GW13040M) ,  Treatment for metabolic diseases,nutritional diseases.  (GD05030F) ,  Drug therapy(=pharmacotherapy)for gynecologic and obstetrical diseases  (GS05020N)

Substance index (1)： Bezafibrate

Reference (30)：

• Przyrembel H, Wendel U, Becker K, Bremer HJ, Bruinvis L, Ketting D, et al. Glutaric aciduria type II: report on a previously undescribed metabolic disorder. Clin Chim Acta 1976;66:227-39.
• Frerman FE, Goodman SI. Deficiency of electron transfer flavoprotein or electron transfer flavoprotein:ubiquinone oxidoreductase in glutaric acidemia type II fibroblasts. Proc Natl Acad Sci USA 1985;82:4517-20.
• Goodman SI, Frerman FE. Glutaric acidaemia type II (multiple acyl-CoA dehydrogenation deficiency). J Inherit Metab Dis 1984;7:33-7.
• Sweetman L, Nyhan WL, Tauner DA, Merritt TA, Singh M. Glutaric aciduria type II. J Pediatr 1980;96:1020-6.
• Lehnert W, Wendel U, Lindenmaier S, Bohm N. Multiple acyl-CoA dehydrogenation deficiency (glutaric aciduria type II), congenital polycystic kidneys, and symmetric warty dysplasia of the cerebral cortex in two brothers. I. Clinical, metabolical, and biochemical findings. Eur J Pediatr 1982;139:56-9.

Terms in the title (5)： 評価 ,  グルタル酸血症 ,  患者 ,  線維芽細胞 ,  ベザフィブラート