Astrocytic hyaline inclusions contain advanced glycation endproducts in familial amyotrophic lateral sclerosis with superoxide dismutase 1 gene mutation: Immunohistochemical and immunoelectron microscopical analyses

Shinsuke Kato, Seikoh Horiuchi, Kenji Nakashima, Asao Hirano, Noriyuki Shibata, Imaharu Nakano, Masaya Saito, Masako Kato, Kohtaro Asayama, Eisaku Ohama

Research output: Contribution to journalArticle

25 Scopus citations

Abstract

To clarify the neuropathological significance of the deposition of NE-carboxymethyl lysine (CML), an advanced glycation endproduct, in astrocytic hyaline inclusions in familial amyotrophic lateral sclerosis (FALS), autopsy specimens from five members of two different families who had the superoxide dismutase 1 (SOD1) gene mutations were analysed. Immunohistochemically most of the neuronal and astrocytic hyaline inclusions were intensely stained by the antibody against CML. The distributions and intensities of the immunoreactivities for CML and SOD1 were similar in the inclusions in both cell types. Immunoelectron microscopy showed that both inclusions consisted of CML-positive granule-coated fibrils and granular materials. No significant CML or SOD1 immunoreactivity was observed in the neurons and astrocytes of the normal control subjects. Our results suggest that astrocytic hyaline inclusions contain CML and SOD1 in FALS patients with SOD1 gene mutations, and that the formation of CML-modified protein (probably CML-modified SOD1) is related to the cell degeneration.

Original languageEnglish (US)
Pages (from-to)260-266
Number of pages7
JournalActa neuropathologica
Volume97
Issue number3
DOIs
StatePublished - Mar 1 1999

Keywords

  • Advanced glycation endproducts
  • Amyotrophic lateral sclerosis
  • Astrocytic hyaline inclusions
  • Nε-carboxymethyl lysine
  • Superoxide dismutase 1

ASJC Scopus subject areas

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

Fingerprint Dive into the research topics of 'Astrocytic hyaline inclusions contain advanced glycation endproducts in familial amyotrophic lateral sclerosis with superoxide dismutase 1 gene mutation: Immunohistochemical and immunoelectron microscopical analyses'. Together they form a unique fingerprint.

  • Cite this