Autoradiographic demonstration of the copper-accumulating tissues in mice with a defect homologous to Menkes' Kinky Hair disease.

Mutated Brindled mice have a genetic defect homologous to the human disorder Menkes' or Kinky Hair disease. These animals were used to survey the tissues in which copper accumulates as result of this defect. Heterozygous and unaffected animals were i.p. injected with radio-copper (64Cu). Sites of pathological 64Cu accumulation were visualized by whole body autoradiography. Apart from the tissues already known to accumulate copper, particularly the kidneys, pathological accumulations were also found in a number of other tissues. It is concluded that in Menkes' disease copper accumulates in epithelioid cells.