Immunohistochemical Studies on Cerebellar Purkinje Cells of Patients with Menkes' Kinky Hair Disease

Shinsuke Kato, Masayuki Ito, Eisaku Ohama, Katsuhiko Mikoshiba, Nobuaki Maeda, Shu‐Hui ‐H Yen, Asao Hirano, Fritz Herz, Masayuki Shintaku

Research output: Contribution to journalArticle

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Abstract

Immunohistochemical investigations were carried out on cerebellar Purkinje cells of eight patients with Menkes' kinky hair disease (MD) using an antibody against the inositol 1, 4, 5‐triphosphate receptor which is identical to the cerebellar Purkinje cell glycoprotein P400 (P400/IP3R). In normal cerebellar Purkinje cells, the cell bodies, axons and dendrites including spiny branchlets were specifically stained by the antibody. By comparison, the immunoreactivity of the MD Purkinje cells ranged from negative to positive. The most frequent staining pattern in the MD Purkinje cells was that of cell bodies and proximal portions of the dendrites expressing P400/IP3R in varying degrees, with the peripheral dendrites including spiny branchlets not being stained. The Purkinje cells of the eight patients were also analyzed for expression of phosphorylated neurofilament proteins (pNFP), αB‐crystallin, stress‐response protein (srp) 72, srp 27 and ubiquitin. Most MD Purkinje cell bodies reacted with the antibody against pNFP. Although the proportion of stained Purkinje cells was less, a positive reaction was also observed with the antibodies to αB‐crystallin, srp 72 and srp 27. By contrast, no normal Purkinje cell was stained by these antibodies. Our results suggest that the function of P400/IP3R in the Purkinje cells of MD patients is impaired, that these cells have an aberrant phosphorylation of NFP and that some of the imparied Purkinje cells synthesize specific sets of stress‐related proteins.

Original languageEnglish (US)
Pages (from-to)159-166
Number of pages8
JournalNeuropathology
Volume13
Issue number2
DOIs
StatePublished - 1993
Externally publishedYes

Fingerprint

Menkes Kinky Hair Syndrome
Purkinje Cells
Dendrites
Antibodies
Neurofilament Proteins
Proteins
Inositol
Ubiquitin
Axons
Glycoproteins

Keywords

  • 4
  • 5‐triphosphate receptor
  • immunohistochemistry
  • inositol 1
  • kinky hair disease
  • Menkes
  • P protein
  • phosphorylated neurofilament proteins
  • Purkinje cell
  • stress‐response proteins

ASJC Scopus subject areas

  • Pathology and Forensic Medicine
  • Clinical Neurology

Cite this

Kato, S., Ito, M., Ohama, E., Mikoshiba, K., Maeda, N., Yen, SH. H., ... Shintaku, M. (1993). Immunohistochemical Studies on Cerebellar Purkinje Cells of Patients with Menkes' Kinky Hair Disease. Neuropathology, 13(2), 159-166. https://doi.org/10.1111/j.1440-1789.1993.tb00227.x

Immunohistochemical Studies on Cerebellar Purkinje Cells of Patients with Menkes' Kinky Hair Disease. / Kato, Shinsuke; Ito, Masayuki; Ohama, Eisaku; Mikoshiba, Katsuhiko; Maeda, Nobuaki; Yen, Shu‐Hui ‐H; Hirano, Asao; Herz, Fritz; Shintaku, Masayuki.

In: Neuropathology, Vol. 13, No. 2, 1993, p. 159-166.

Research output: Contribution to journalArticle

Kato, S, Ito, M, Ohama, E, Mikoshiba, K, Maeda, N, Yen, SHH, Hirano, A, Herz, F & Shintaku, M 1993, 'Immunohistochemical Studies on Cerebellar Purkinje Cells of Patients with Menkes' Kinky Hair Disease', Neuropathology, vol. 13, no. 2, pp. 159-166. https://doi.org/10.1111/j.1440-1789.1993.tb00227.x
Kato, Shinsuke ; Ito, Masayuki ; Ohama, Eisaku ; Mikoshiba, Katsuhiko ; Maeda, Nobuaki ; Yen, Shu‐Hui ‐H ; Hirano, Asao ; Herz, Fritz ; Shintaku, Masayuki. / Immunohistochemical Studies on Cerebellar Purkinje Cells of Patients with Menkes' Kinky Hair Disease. In: Neuropathology. 1993 ; Vol. 13, No. 2. pp. 159-166.
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