TY - JOUR
T1 - Pathological characterization of astrocytic hyaline inclusions in familial amyotrophic lateral sclerosis
AU - Kato, Shinsuke
AU - Hayashi, Hiroko
AU - Nakashima, Kenji
AU - Nanba, Eiji
AU - Kato, Masako
AU - Hirano, Asao
AU - Nakano, Imaharu
AU - Asayama, Kohtaro
AU - Ohama, Eisaku
PY - 1997
Y1 - 1997
N2 - 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.
AB - 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.
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M3 - Article
C2 - 9273821
AN - SCOPUS:0030797166
SN - 0002-9440
VL - 151
SP - 611
EP - 620
JO - American Journal of Pathology
JF - American Journal of Pathology
IS - 2
ER -