Effects of Copper and Hydrocortisone on Cell Cycle of Cultured Fibroblasts from the Macular Mouse as a Model of Menkes Disease: DNA Analysis by Flow Cytometry

Abstract Effects of copper and hydrocortisone on the proliferation of cultured fibroblasts from macular mouse, a model of Menkes kinky hair disease, were analyzed by flow cytometry. In fibroblasts from the macular mouse, copper induced cytotoxicity and S-phase accumulation of cell cycle were observed at lower concentrations of copper than in fibroblasts from the controls. However, the fibroblasts cultured with a combination of copper and hydrocortisone revealed recruitment of cell cycle. These results suggest that the proliferative activity of the cells from a macular mouse are more vulnerable to an excess amount of copper than normal, and that supplementation of hydrocortisone is effective for the Menkes kinky hair disease.