Immunohistochemical detection of products of lipidperoxidation and protein glycation in the cerebellumof Menkes' kinky hair disease patients

摘要

Menkes' kinky hair disease (MKHD) is a hereditary Cu malabsorption disorder characterized by hair abnormalities, mental retardation and hypotonia. We previously suggested that Cu depletion induces oxidative stress generation in the cerebellum affected with this disease. To test oxidative damage involvement in MKHD, we analyzed immunohistochemical localization of products of lipid peroxidation and protein glycation in the cerebellum of five MKHD patients and five age-matched controls. Immunoreactivities for these examined products were undetectable in the control cases, and were localized in Purkinje cells, glial cells and endothelial cells of the MKHD patients. Our results indicate a certain implication for both lipid peroxidation and protein glycoxidation in MKHD.