April 1981 in “Pediatric research” This study found that abnormal liver and kidney copper metabolism in Br females had no clinical effects, whereas defective brain copper metabolism in Br males was clinically significant.
40 citations
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July 1981 in “Journal of Neuropathology & Experimental Neurology” In this study, brindled mutant mice with clinical features similar to kinky hair syndrome showed marked improvement and prevention of neuronal degeneration after receiving cupric chloride injections, with timing of administration being crucial.
23 citations
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January 1985 in “Journal of Neuropathology & Experimental Neurology” This study found that cupric chloride treatment may partially correct delayed maturation and abnormal arborization of Purkinje cells in the cerebellum of hemizygous brindled mice, a model for Kinky hair disease.
11 citations
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November 1991 in “Journal of Neuropathology & Experimental Neurology” This study found that brindled mottled mice, a model of Kinky hair syndrome, exhibited abnormal development of catecholamine neurons, with increased TH-immunoreactive neurons and altered neurochemical profiles compared to controls.
16 citations
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June 1983 in “Journal of Neurochemistry” This study found that copper injections increased dopamine-β-hydroxylase activity in the brains of mottled mice, an animal model for Menkes' syndrome.