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- Title
Cerebellar superoxide dismutase expression in Menkes' kinky hair disease: an immunohistochemical investigation.
- Authors
Shibata, Noriyuki; Hirano, Asao; Kobayashi, Makio; Umahara, Takahiko; Kawanami, Toru; Asayama, Kohtaro
- 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.
- Publication
Acta Neuropathologica, 1995, Vol 90, Issue 2, p198
- ISSN
0001-6322
- Publication type
Article
- DOI
10.1007/BF00294321