Pathological characterization of astrocytic hyaline inclusions in familial amyotrophic lateral sclerosis

Shinsuke Kato, Hiroko Hayashi, Kenji Nakashima, Eiji Nanba, Masako Kato, Asao Hirano, Imaharu Nakano, Kohtaro Asayama, Eisaku Ohama

Research output: Contribution to journalArticlepeer-review

97 Scopus citations

Abstract

To clarify the pathological characteristics of astrocytic hyaline inclusions (Ast-HIs) in patients with familial amyotrophic lateral sclerosis (FALS) with neuronal Lewy-body-like hyaline inclusions (LBHIs), eight autopsies on members of four different families, including two long-term surviving patients with clinical courses of over 10 years, were analyzed. Ast-HIs were found only in the two long-term surviving patients who belonged to different families and to different races. Ast-HIs were ultrastructurally composed of 15- to 25-nm granule-coated fibrils that had immunoreactivities to superoxide dismutase 1 (SOD1) and ubiquitin. Approximately 50% of the Ast- HIs expressed αB-crystallin, metallothionein, glutamine synthetase, and tubulin (α and β) at various intensities. Some Ast-HIs reacted with antibodies to tau protein, S-100 protein, and heat shock protein 27. The Ast- HIs were not stained for glial fibrillary acidic protein. Our results suggest a cooperative role of superoxide dismutase 1, ubiquitin, and cytoskeletal proteins in the formation of granule-coated fibrils (namely, Ast-HIs) and provide evidence that Ast-HIs are formed in certain long-surviving familial amyotrophic lateral sclerosis patients with neuronal Lewybody-like hyaline inclusions.

Original languageEnglish (US)
Pages (from-to)611-620
Number of pages10
JournalAmerican Journal of Pathology
Volume151
Issue number2
StatePublished - Aug 19 1997

ASJC Scopus subject areas

  • Pathology and Forensic Medicine

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