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

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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 1999

Fingerprint

Hyalin
Mutation
Genes
Immunoelectron Microscopy
Amyotrophic lateral sclerosis 1
N(6)-carboxymethyllysine
Superoxide Dismutase-1
Astrocytes
Autopsy
Neurons
Antibodies

Keywords

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

ASJC Scopus subject areas

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

Cite this

Astrocytic hyaline inclusions contain advanced glycation endproducts in familial amyotrophic lateral sclerosis with superoxide dismutase 1 gene mutation : Immunohistochemical and immunoelectron microscopical analyses. / Kato, Shinsuke; Horiuchi, Seikoh; Nakashima, Kenji; Hirano, Asao; Shibata, Noriyuki; Nakano, Imaharu; Saito, Masaya; Kato, Masako; Asayama, Kohtaro; Ohama, Eisaku.

In: Acta Neuropathologica, Vol. 97, No. 3, 03.1999, p. 260-266.

Research output: Contribution to journalArticle

Kato, Shinsuke ; Horiuchi, Seikoh ; Nakashima, Kenji ; Hirano, Asao ; Shibata, Noriyuki ; Nakano, Imaharu ; Saito, Masaya ; Kato, Masako ; Asayama, Kohtaro ; Ohama, Eisaku. / Astrocytic hyaline inclusions contain advanced glycation endproducts in familial amyotrophic lateral sclerosis with superoxide dismutase 1 gene mutation : Immunohistochemical and immunoelectron microscopical analyses. In: Acta Neuropathologica. 1999 ; Vol. 97, No. 3. pp. 260-266.
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