Pathological characteristics of astrocytic hyaline inclusions in familial amyotrophic lateral

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To clarify the pathological characteristics of astrocytic hyaline inclusions (Ast-HI) in patients with familial amyptrophic lateral sclerosis (FALS) with the superoxide dismutase 1 (SOD1) genemutations, eight autopsies on members of four different families were analyzed. The neuronal Lewy body-like hyaline inslusion(LBHI) was also a characteristic neuropathological marker of the mutant SOD1-related FALS.Both Ast-Ill and LBHI were ultrastructurally composed of approximately 15- to 25-nm granule-coated fibrils that had immunoreactivities to SOD1 and N^<epsilon> -carboxymethyl lysine (CML, an advanced glycation endproduct). These results suggest that Ast-HI and LBHI contain SOD1 and CML, and that the formation of CML-modified SOD1 (probably CML-modified mutant SOD 1) is related to the cell degeneration.