Effect of copper on Menkes' and normal cultured skin fibroblasts

N. G. Beratis, M. Yee, G. U. LaBadie, K. Hirschhorn

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9 Scopus citations


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 μg 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 μg CuCl2/ml. Normal cells showed a reduction in DNA synthesis at CuCl2 concentrations greater than 50 μg/ml, whereas at concentrations between 2 and 30 μg/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.

Original languageEnglish
Pages (from-to)305-317
Number of pages13
JournalDevelopmental Pharmacology and Therapeutics
Issue number5
StatePublished - 1980
Externally publishedYes


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