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Effect of Copper on Cultured Fibroblasts from Macular Mouse as a Model of Menkes Kinky Hair Disease
Author(s) -
KATSURA Tadahiko,
YAMANO Tsunekazu,
SHIMADA Morimi
Publication year - 1987
Publication title -
congenital anomalies
Language(s) - English
Resource type - Journals
eISSN - 1741-4520
pISSN - 0914-3505
DOI - 10.1111/j.1741-4520.1987.tb00708.x
Subject(s) - fibroblast , menkes disease , dermal fibroblast , cell culture , biology , microbiology and biotechnology , copper , chemistry , genetics , copper metabolism , organic chemistry
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.