Copper metabolism in mottled mouse mutants: copper therapy of brindled (Mobr) mice

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Abstract
Cu therapy was applied to brindled mouse mutants [models of Menkes syndrome], which suffer from lethal hypocupremia, by using cuprous and cupric solutions. The method of treatment was a single s.c. injection of 50 .mu.g of Cu at 7 days of age. Early effects of the dose were: prevention of the tremors and spasms seen in untreated mutants, raising to normal and near-normal of ceruloplasmin oxidase and lysyl oxidase activities and pigmentation of skin and fur. Growth of mutants was retarded up to 23 days of age, but thereafter they rapidly gained weight to be nearly normal by 60 days of age. At 3 days after injection, as high as those of treated normals, which had remained unchanged. Cu in mutant livers had increased only slightly compared with the normal control. A state of Cu deficiency recurred in mutant tissues by 25 days after injection. A solution of Cu+, retained as such by an alkyl polyether and sebacic acid resulted in greater growth rates after 23 days than did 3 other Cu treatments. Cu+ may have resulted in an improved growth response because it is more readily metabolized than Cu2+. Delayed response of Cu from the site of injection may have played an important role.