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 20/118）[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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ConclusionsA combination therapy comprising copper injection and oral disulfiram improved copperconcentrations in serum, liver, and brain, and enhanced cytochrome c oxidase activity andcatecholamine m....
ConclusionsA combination therapy comprising copper injection and oral disulfiram improved copperconcentrations in serum, liver, and brain, and enhanced cytochrome c oxidase activity andcatecholamine metabolism in the brain of the macular mouse, a model of MD. These results suggestthat the lipophilic copper-disulfiram complex can penetrate cellular membranes, including theblood-brain barrier and Golgi membranes, in MD-affected cells, thereby making copper available tocopper-dependent enzymes. Connective tissue disorders, including arterial abnormalities, bladderdiverticula, and osteoporosis, are also serious problems in patients with MD. These disorders arecaused by reduced activity of lysyl oxidase, a secretory copper-dependent enzyme. Thus, thecopper-disulfiram combination therapy may also be effective in treating connective tissue disordersassociated with MD.AcknowledgementThis work was in part by a Grant of Research on Intractable Diseases from Ministry of Health,Labour and Welfare of Japan (23-326) and a memorial fund for Naoki, a former patient withMenkes disease.References[1] Kodama H, Fujisawa C. Copper metabolism and inherited copper transport disorders: molecularmechanisms, screening, and treatment. Metallomics 2009; 1:42-52.[2] Kodama H, Meguro Y, Abe T, Rayner MH, Suzuki KT, Kobayashi S, Nishimura M. Geneticexpression of Menkes disease in cultured astrocytes of the macular mouse. J Inherited Metab Dis1991; 14:896-901.[3] Kodama H. Recent developments in Menkes disease. J Inherited Metab Dis 1993; 16:791-9.[4] Qian Y, Tiffany-Castigloini E, Welsh J, Harris ED. Copper efflux from murine microvascularcells requires expression of the Menkes disease Cu-ATPase. J Nutr 1998; 128:1276-82.