Some aspects of copper metabolism in Brindled mice

The semi-dominant X-linked mutation in Brindled mice causes a severe copper deficiency of which the hemizygous Brindled mice die between 14 and 21 days post partum. Previously, in analogy to Menkes' disease in man, the primary defect in mutated Brindled mice has been described as a block in the resorption of alimentary copper, i.e., the transport of copper from the intestinal lumen into the portal blood circulation. During this research it became clear that the impaired resorption of alimentary copper is only a part of a more general aberration of copper metabolism in epithelioid cells. Tracer techniques using 64Cu are used for metabolism studies. (Auth.)