The Effect of Copper Supplementation on the Brindled Mouse

The study on brindled mottled mice, a model for kinky hair syndrome in humans, found that copper supplementation via cupric chloride injections significantly improved clinical symptoms and prevented neuronal degeneration. The timing of the injection was crucial, with the most effective results observed when administered on postnatal days 7 to 10. Mice receiving injections on these days overcame lethality and showed no neuronal degeneration, although some mitochondrial changes persisted. Additional injections further reduced abnormalities, particularly in the cerebellum. These findings suggested that copper supplementation was beneficial for clinical improvement and prevention of neuropathological lesions in these mutant mice.