Ubiquitin and phosphorylated neurofilament epitopes in ballooned neurons of the extraocular muscle nuclei in a case of Werdnig-Hoffmann disease

S. Kato, A. Hirano

Research output: Contribution to journalArticle

27 Citations (Scopus)

Abstract

The extraocular muscle nuclei in one case of Werdnig-Hoffmann disease were examined immunocytochemically using antibodies against phosphorylated neurofilament (pNF) and ubiquitin (UBQ). The oculomotor and trochlear nuclei showed several chromatolytic ballooned neurons. All ballooned neurons contained epitopes of pNF and UBQ. pNF were present mainly in the periphery of the cell in a ring-like shape and were occasionally seen in the center of some cells. On the other hand, the structures stained by the antibody to UBQ were small vesicles or granules and most of them were aggregated in the center of the cell. These distribution patterns of pNF and UBQ may be unique in Werdnig-Hoffmann disease, since similar patterns were reported in other types of neurons of Werdnig-Hoffmann disease but were not seen in two other motor neuron diseases: classical amyotrophic lateral sclerosis, and familial amyotrophic lateral sclerosis with posterior column and spinocerebellar tract involvement.

Original languageEnglish (US)
Pages (from-to)334-337
Number of pages4
JournalActa Neuropathologica
Volume80
Issue number3
DOIs
StatePublished - Jul 1990

Fingerprint

Oculomotor Muscles
Spinal Muscular Atrophies of Childhood
Intermediate Filaments
Ubiquitin
Epitopes
Neurons
Spinocerebellar Tracts
Tegmentum Mesencephali
Motor Neuron Disease
Antibodies

Keywords

  • Chromatolytic neuron
  • Extraocular muscle nuclei
  • Phosphory-lated neurofilament
  • Ubiquitin
  • Werdnig-Hoffmann disease

ASJC Scopus subject areas

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

Cite this

Ubiquitin and phosphorylated neurofilament epitopes in ballooned neurons of the extraocular muscle nuclei in a case of Werdnig-Hoffmann disease. / Kato, S.; Hirano, A.

In: Acta Neuropathologica, Vol. 80, No. 3, 07.1990, p. 334-337.

Research output: Contribution to journalArticle

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