Immunocytochemical and ultrastructural studies of hyaline inclusions in sporadic motor neuron disease.

摘要: We investigated hyaline inclusion bodies (HI) immunocytochemically and ultrastructurally in six cases of sporadic motor neuron disease (MND). All HI contained large amounts ubiquitin some were stained at the core or center with anti-neurofilament antibody, surrounding halo unstained. No antibodies raised against cytoskeletal proteins such as high-molecular weight microtubule-associated phosphorylated tau. Ultrastructurally, chiefly composed filaments measuring about 20 nm diameter thicker than neurofilaments, fine granules frequently one more four characteristic profiles, i.e., small electron-dense materials resembling Bunina bodies, bundles tubular approximately diameter, cores, focal accumulations randomly arranged neurofilaments. Hyaline inclusions can be regarded markers for MND well familial amyotrophic lateral sclerosis. have a markedly heterogeneous ultrastructure and, therefore, differences immunoreactivity antineurofilament are not unexpected.