Abstract
X-linked murine mutant Br is a model for Menkes Kinky Hair Synd., a disorder of Cu metabolism with no effective post-natal Rx. To assess response to prenatal Rx, pregnant dams received Cu, 35 mcg/kg/d S.C., last 1-15d of pregnancy. This resulted in reduced litter size and decreased viability of newborn mice. Prenatal Cu-Rx also did not increase affect tissue Cu.
Normal (nl) mice born to n1 and Br heterozygote mothers were sacrificed immediately after birth and brain, liver, kidney and placental Cu determined for comparison with values in Br male hemizygotes. The brain and liver Cu in Br males was lower than nl; the kidney Cu was higher than nl; the placental Cu was approximately twice nl. No differences were found in the Cu contents of nl mice born to n1 vs. carriers. The 1st wk of life, wgt increased in n1 3-5xb.w. vs. 2-3xb.w. in the Br males. Brain Cu content in nl remains stable, while brain Cu in Br males decreased to approx. 1/4 nl. Neither wgt gain nor brain, liver, kidney Cu at the end of 1st wk of life were altered by prenatal Rx; only placental Cu increased. Offspring of mothers Cu-Rx during pregnancy were then Cu-loaded at age 7d: brain, liver and kidney Cu did not increase in n1; liver Cu in .Br males increased slightly, not to levels comparable to age-matched n1, but kidney Cu increased significantly. From these data we conclude that response to Cu-Rx in the young Br male is analagous to that of the infant with Menkes Synd. In addition, prenatal Rx is not effective and may be harmful.
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Garnica, A., Chang, S. 1124 PERINATAL COPPER (Cu) TREATMENT (Rx) IN BRINDLED (Br) MICE. Pediatr Res 15 (Suppl 4), 630 (1981). https://doi.org/10.1203/00006450-198104001-01150
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DOI: https://doi.org/10.1203/00006450-198104001-01150