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Histochemical localization of copper in the intestine and kidney of macular mice: light and electron microscopic
1Department of Pediatrics, Teikyo University School of Medicine, Tokyo, Japan.
Abstract:
Menkes' disease is an X-linked recessive disorder characterized by accumulation of copper in various organs and cells, such as the intestine, kidney, and cultured fibroblasts. Light and electron microscopic localization of Cu was investigated in the intestine and kidney of macular mice, an animal model of Menkes' disease, by a modified sulfide-silver method. Cu was accumulated in the cytoplasm of the absorptive epithelial cells, the vascular endothelium, and secretory granules of the Paneth cells. In kidney the distal tubule cells and glomeruli of both macular and control mice stained faintly, whereas the organelle-free cytoplasm in the proximal tubule cells of macular mice stained more intensely than those of controls. The nuclei, mitochondria, and lysosomes of the cells of macular mice hardly stained at all. These findings indicate that Cu is concentrated in the organelle-free cytoplasm of the affected cells of macular mice. This suggests that the Menkes' mutation affects Cu transport from the cytosol to the organelles in the cell.
Insights
Menkes
Area of Science:
- Cell Biology
- Genetics
- Biochemistry
Background:
- Menkes' disease is an X-linked recessive disorder.
- It involves copper accumulation in various organs and cells.
- Macular mice serve as an animal model for studying Menkes' disease.
Purpose of the Study:
- To investigate the cellular and subcellular localization of copper (Cu) in the intestine and kidney of macular mice.
- To understand the impact of the Menkes' mutation on copper distribution within cells.
Main Methods:
- Utilized a modified sulfide-silver method for light and electron microscopic localization of copper.
- Examined copper distribution in the intestine and kidney tissues of macular and control mice.
Main Results:
- Copper accumulated in the cytoplasm of intestinal absorptive epithelial cells, vascular endothelium, and Paneth cell secretory granules.
- In the kidney, proximal tubule cells of macular mice showed intense copper staining in organelle-free cytoplasm compared to controls.
- Nuclei, mitochondria, and lysosomes showed minimal copper staining in affected cells.
Conclusions:
- Copper is concentrated in the organelle-free cytoplasm of affected cells in macular mice.
- The Menkes' mutation likely impairs copper transport from the cytosol into cellular organelles.