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Effect of copper on Menkes' and normal cultured skin fibroblasts
Abstract:
Skin fibroblasts from normal subjects and from patients with Menkes' disease did not demonstrate any significant differences in morphologic or growth characteristics when cultured in medium without added copper. Addition of CuCl2 to the medium caused a reduction in the growth rate of the fibroblasts. Menkes' fibroblasts were more sensitive to copper than normal cells. Concentrations greater than 50 micrograms CuCl2/ml of medium caused extensive cell death especially in the mutant cells. The rate of DNA synthesis in Menkes' fibroblasts was reduced in the presence of 2--100 micrograms CuCl2/ml. Normal cells showed a reduction in DNA synthesis at CuCl2 concentrations greater than 50 micrograms/ml, whereas at concentrations between 2 and 30 micrograms/ml the rate of DNA synthesis was increased. These findings indicate that CuCl2 is more toxic to Menkes' than to normal fibroblasts and suggest that a similar response of neuronal cells may occur in the patients in vivo.
Insights
Menkes
Area of Science:
- Cell Biology
- Genetics
- Biochemistry
Background:
- Menkes' disease is a rare genetic disorder affecting copper metabolism.
- Understanding cellular responses to copper is crucial for Menkes' disease research.
Purpose of the Study:
- To investigate the differential sensitivity of Menkes' disease fibroblasts to copper.
- To explore the impact of copper on fibroblast growth and DNA synthesis.
Main Methods:
- Culturing skin fibroblasts from normal subjects and Menkes' disease patients.
- Assessing cell morphology, growth rates, and DNA synthesis in varying copper concentrations (CuCl2).
Main Results:
- Menkes' fibroblasts exhibited increased sensitivity and cell death at lower copper concentrations compared to normal cells.
- Copper (CuCl2) reduced DNA synthesis in Menkes' fibroblasts at lower concentrations than in normal fibroblasts.
- Normal fibroblasts showed increased DNA synthesis at low copper levels, unlike Menkes' cells.
Conclusions:
- Copper(II) chloride (CuCl2) is demonstrably more toxic to Menkes' fibroblasts than to normal fibroblasts.
- These findings suggest that neuronal cells in Menkes' disease patients may exhibit similar copper hypersensitivity in vivo.