Histochemical studies of fibroblasts from patients with Menkes kinky hair disease and Wilson's disease |
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| Authors: | H C Liu W Y Chan and O M Rennert |
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| Institution: | (1) Department of Anatomy, University of Hong Kong, Hong Kong;(2) Department of Pediatrics and Department of Biochemistry and Molecular Biology, University of Oklahoma Health Sciences Center, Oklahoma City, Oklahoma, USA |
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| Abstract: | Summary Cultured fibroblasts from Menkes kinky hair disease patients showed markedly reduced succinate dehydrogenase and amine oxidase activities. Cytochrome oxidase activity, however, was greatly reduced in some cells and almost normal in others. Cultured fibroblasts from patients with Wilson's disease showed moderately reduced succinate dehydrogenase and cytochrome oxidase activities. Amine oxidase activity was only slightly reduced when compared to that of normal. These results indicated that the histochemical phenotype observed in fibroblasts from patients with Menkes kinky hair disease and Wilson's disease were distinctly different from each other and from normal fibroblasts. |
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