Abstract
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.
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Shibata, N., Hirano, A., Kobayashi, M. et al. Cerebellar superoxide dismutase expression in Menkes' kinky hair disease: an immunohistochemical investigation. Acta Neuropathol 90, 198–202 (1995). https://doi.org/10.1007/BF00294321
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DOI: https://doi.org/10.1007/BF00294321