Abstract
Abstract When the fibroblast from a patient with Menkes kinky hair disease (MKHD) was cultured in a copper‐enriched medium, the rounded, detached and floating cells increased in number. Cell proliferation was also inhibited considerably in the fibroblast from MKHD as compared to that from the normal control. The fibroblast from hemizygote of the macular mouse showed a similar sensitivity for copper in culture medium. Copper content was significantly increased in the cultured fibroblasts from the patient with MKHD and the hemizygote of the macular mouse in a copper‐enriched medium compared to that in the fibroblast from the respective control. These results were well identical to those in literatures concerning MKHD and indicate that the fibroblast from the hemizygote of the macular mouse biochemically show a close similarity to those from MKHD and that the macular mouse serves cellbiologically as a model of MKHD.
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