厚生労働科学研究費補助金（難治性疾患克服研究事業） 「Menkes 病・occipital horn 症候群の実態調査、早期診断基準確立、治療法開発に関する研究」 平成23年度 総括・分担研究報告書（page 15/118）[厚生労働科学研究費補助金（難治性疾患克服研究事業） 「Menkes 病・occipital horn 症候群の実態調査、早期診断基準確立、治療法開発に関する研究」 平成23年度 総括・分担研究報告書]
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Effect of Copper and Disulfiram Combination Therapy on the Macular Mouse, a Model ofMenkes DiseaseWattanaporn Bhadhprasit a , Hiroko Kodama a,b? , Chie Fujisawa a , Tomoko Hiroki a , EishinOgawa aaDep....
Effect of Copper and Disulfiram Combination Therapy on the Macular Mouse, a Model ofMenkes DiseaseWattanaporn Bhadhprasit a , Hiroko Kodama a,b? , Chie Fujisawa a , Tomoko Hiroki a , EishinOgawa aaDepartment of Pediatrics, Teikyo University School of Medicine, TokyobDepartment of Health and Dietetics, Faculty of Health and Medical Sciences, Teikyo HeiseiUniversity, TokyoShort title: Therapy with copper and disulfram on macular miceABSTRACTMenkes disease (MD) is a genetic neurodegenerative disorder characterized by copper deficiencydue to a defect in ATP7A. Standard treatment involves parenteral copper-histidine administration.However, the treatment is ineffective if initiated after two months of age, because the administeredcopper accumulates in the blood-brain barrier and is not transported to neurons. To resolve thisissue, we investigated the effects of a combination therapy comprising copper and disulfiram, alipophilic chelator, in the macular mouse, an animal model of MD. Seven-day-old macular micetreated subcutaneously with 50μg of CuCl2 on postnatal day 4 were used. The mice were given asubcutaneous injection of CuCl2 (10μg) with oral administration of disulfiram (0.3 mg/g bodyweight) twice a week until eight weeks of age, and then sacrificed. Copper concentrations in thecerebellum, liver, and serum of treated macular mice were significantly higher than those of controlmacular mice, which received only copper. Mice treated with the combination therapy exhibitedhigher cytochrome c oxidase activity in the brain. The ratios of noradrenaline and adrenaline todopamine in the brain were also increased by the treatment, suggesting that dopamineβ-hydroxylase activity was improved by the combination therapy. Liver and renal functions were