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 17/118）[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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been used for the treatment of alcoholism and cocaine addiction and as a modulator ofcisplatin-induced toxicity [8?10]; thus, oral disulfiram is easily applicable in the clinical setting.Here, we repo....

been used for the treatment of alcoholism and cocaine addiction and as a modulator ofcisplatin-induced toxicity [8?10]; thus, oral disulfiram is easily applicable in the clinical setting.Here, we report the effects of a combination therapy comprising copper injection and oraldisulfiram on the macular mouse, an animal model of MD [11].Materials and methodsAnimalsMale hemizygous macular mice and normal littermate controls were treated with a singlesubcutaneous injection of cupric chloride solution (50μg of CuCl2) on postnatal day 4, becausemacular mice die without this treatment. All mice were maintained under standard conditions.Macular mice were separated into control and treated groups. The latter group was treated with asubcutaneous injection of CuCl2 (10μg) and oral administration of disulfiram (0.3 mg/g bodyweight) twice a week from the age of 7 days to 8 weeks, and then sacrificed. Control mice weregiven a subcutaneous injection of CuCl2 (10μg) as above, but disulfiram was replaced with doubledistilled water. Normal littermates were used as normal controls. Body weights were measuredtwice a week during the treatment period. The cerebrum, cerebellum, kidney, liver, and intestineswere dissected. Sera and tissues were stored at ?80°C until analysis. This study was approved byTeikyo University School of Medicine Animal Ethics Committee (07-035).Measurement of copper concentrationTissue samples were dried at 120°C for 12 h and wet-digested with concentrated HNO3 byheating at 120°C, and the resultant residues were dissolved in 2 mol/L HNO3. Serum samples werewet-digested with concentrated HNO3 by heating at 120°C. Copper concentration was analyzedwith a Hitachi Z-8100 atomic absorption spectrophotometer (Hitachi Industries, Japan). Allglassware was washed with nitric acid to avoid metal contamination.