Cerebellar superoxide dismutase expression in Menkes' kinky hair disease: an immunohistochemical investigation.
This comparative immunohistochemical study deals with the expression of the cytosolic Cu/Zn-binding and mitochondrial Mn-dependent superoxide dismutases (SODs) in the cerebella of five patients with Menkes' kinky hair disease (MKHD) and five age-matched controls. Several cell types, including Purkinje cells and reactive astrocytes, of all MKHD patients examined were intensely stained by an antibody to Mn SOD, but not by an anti-Cu/Zn SOD antibody. By contrast, the cells of the five controls reacted very weakly or not at all with the anti-Mn SOD antibody, but were strongly reactive with the antibody to Cu/Zn SOD. These results suggest that the increased Mn SOD immunoreactivity in MKHD reflects enzyme induction as a protective mechanism against the highly toxic superoxide anion generated under the disease conditions.
D000293:Adolescent; D000328:Adult; D002531:Cerebellum / Q000473:pathology*; D002540:Cerebral Cortex / Q000276:immunology; D006801:Humans; D007150:Immunohistochemistry; D007706:Menkes Kinky Hair Syndrome / Q000276:immunology* / Q000473:pathology*; D011689:Purkinje Cells / Q000648:ultrastructure; D013482:Superoxide Dismutase / Q000096:biosynthesis* / Q000378:metabolism
D013482:Superoxide Dismutase