Abstract
The X-linked mouse mutant brindled is a model for Menkes syndrome. As such, young hemizygotes and heterozygotes demonstrate low hepatic copper contents and serum ceruloplasmin concentrations, which cannot be corrected with parenteral copper. In adult brindled animals ceruloplasmin and hepatic copper are not significantly different from those in normal animals. Measurement of copper concentrations in hepatic subcellular fractions of young brindled animals demonstrates reduced copper concentrations in all fractions but especially in mitochondrial and nuclear fractions. Parenteral copper, 10 mcg/g/day x 5 days, as cupric chloride solution, in young control animals results in increased copper concentrations predominantly in nuclear, mitochondrial, and supernatant fractions. In contrast, identical treatment of young brindled animals demonstrates increases in copper concentration primarily in the supernatant fraction. No difference was observed in copper concentrations of subcellular fractions from similarly treated adult brindled heterozygotes when compared with treated controls. These data implicate a defect in hepatic copper retention in brindled young, predominantly involving the nuclear and mitochondrial fractions, associated with impaired copper incorporation into ceruloplasmin.
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Garnica, A., Bates, J. & Rennert, O. DEFECTIVE HEPATIC COPPER STORAGE IN THE BRINDLED MOUSE. Pediatr Res 21 (Suppl 4), 290 (1987). https://doi.org/10.1203/00006450-198704010-00736
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DOI: https://doi.org/10.1203/00006450-198704010-00736