HISTOCHEMICAL-LOCALIZATION OF COPPER IN THE INTESTINE AND KIDNEY OF MACULAR MICE - LIGHT AND ELECTRON-MICROSCOPIC STUDY

Menkes' disease is an X-linked recessive disorder characterized by acc umulation of copper in various organs and cells, such as the intestine , kidney, and cultured fibroblasts. Light and electron microscopic loc alization of Cu was investigated in the intestine and kidney of macula r mice, an animal model of Menkes' disease, by a modified sulfide-silv er method. Cu was accumulated in the cytoplasm of the absorptive epith elial cells, the vascular endothelium, and secretory granules of the P aneth cells. In kidney the distal tubule cells and glomeruli of both m acular and control mice stained faintly, whereas the organelle-free cy toplasm in the proximal tubule cells of macular mice stained more inte nsely than those of controls. The nuclei, mitochondria, and lysosomes of the cells of macular mice hardly stained at all. These findings ind icate that Cu is concentrated in the organelle-free cytoplasm of the a ffected cells of macular mice. This suggests that the Menkes' mutation affects Cu transport from the cytosol to the organelles in the cell.