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 Citations (Scopus)

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 1995

Fingerprint

Menkes Kinky Hair Syndrome
Superoxide Dismutase
Antibodies
Enzyme Induction
Poisons
Purkinje Cells
Superoxides
Astrocytes
Cerebellum

Keywords

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

ASJC Scopus subject areas

  • Neuroscience(all)
  • Pathology and Forensic Medicine
  • Clinical Neurology

Cite this

Cerebellar superoxide dismutase expression in Menkes' kinky hair disease : an immunohistochemical investigation. / Shibata, Noriyuki; Hirano, Asao; Kobayashi, Makio; Umahara, Takahiko; Kawanami, Toru; Asayama, Kohtaro.

In: Acta Neuropathologica, Vol. 90, No. 2, 08.1995, p. 198-202.

Research output: Contribution to journalArticle

Shibata, N, Hirano, A, Kobayashi, M, Umahara, T, Kawanami, T & Asayama, K 1995, 'Cerebellar superoxide dismutase expression in Menkes' kinky hair disease: an immunohistochemical investigation', Acta Neuropathologica, vol. 90, no. 2, pp. 198-202. https://doi.org/10.1007/BF00294321
Shibata, Noriyuki ; Hirano, Asao ; Kobayashi, Makio ; Umahara, Takahiko ; Kawanami, Toru ; Asayama, Kohtaro. / Cerebellar superoxide dismutase expression in Menkes' kinky hair disease : an immunohistochemical investigation. In: Acta Neuropathologica. 1995 ; Vol. 90, No. 2. pp. 198-202.
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