Cerebellar superoxide dismutase expression in Menkes' kinky hair disease: an immunohistochemical investigation

Noriyuki Shibata, Asao Hirano, Makio Kobayashi, Takahiko Umahara, Toru Kawanami, Kohtaro Asayama

Research output: Contribution to journalArticle

21 Scopus citations

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.

Original languageEnglish (US)
Pages (from-to)198-202
Number of pages5
JournalActa Neuropathologica
Volume90
Issue number2
DOIs
StatePublished - Aug 1 1995

Keywords

  • Cerebellum
  • Menkes' kinky hair disease
  • Purkinje cells
  • Reactive astrocytes
  • Superoxide dismutase

ASJC Scopus subject areas

  • Pathology and Forensic Medicine
  • Clinical Neurology
  • Cellular and Molecular Neuroscience

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