Heterogeneous inclusions in neurofilament inclusion disease

Hirotake Uchikado, Ang Li, Wen Lang Lin, Dennis W. Dickson

Research output: Contribution to journalArticle

17 Scopus citations

Abstract

Neurofilament inclusion disease (NFID) is a rare disease, whose pathogenesis remains to be elucidated. Immunoreactivity of ubiquitin-binding protein p62 has been reported in various neurodegenerative diseases, but it has not been studied in NFID. In this report we show p62 immunoreactivity in neuronal perikaryon of three cases of NFID. We found inclusions in NFID to be heterogenous based on immunoreactivity for α-internexin, phosphorylated neurofilament-H, p62 and ubiquitin. Moreover, we showed both p62- and α-internexin-immunoreactive inclusions within the perikarya of the same neuron. Electron microscopy findings support the notion that inclusions in NFID are heterogenous. The present study extends the list of proteins that have been identified as components of neuronal inclusions in NFID, and may help account for the pathogenesis of NFID.

Original languageEnglish (US)
Pages (from-to)417-421
Number of pages5
JournalNeuropathology
Volume26
Issue number5
DOIs
StatePublished - Oct 1 2006

Keywords

  • Neurofilament inclusion disease (NFID)
  • Neuronal intermediate filament inclusion disease (NIFID)
  • Ubiquitin
  • p62
  • α-internexin

ASJC Scopus subject areas

  • Pathology and Forensic Medicine
  • Clinical Neurology

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