オリーブ橋小脳萎縮症における細胞骨格蛋白の異常について

Cytoskeletal Proteins Abnormalities in Olivopontocerebellar Atrophy

　オリーブ橋小脳萎縮症（OPCA）における希突起膠細胞封入体に鋭敏なGallyas鍍銀法を用いて，OPCA 4例，Joseph病4例を含む15例の神経変性疾患，3例の正常対照脳を染色し，細胞骨格蛋白抗体による免疫染色によりOPCA症例の嗜銀性構造物の抗原特性を明らかにした。嗜銀性構造物はアルツハイマー病とOPCA症例にみられた。OPCA症例の嗜銀性構造物は白質では希突起膠細胞に，被殼，橋核，下オリーブ核では神経細胞，星状膠細胞，希突起膠細胞にみられた。橋小脳路，下オリーブ核では中空構造をした嗜銀性神経線維がみられ，髄鞘の残存している神経線維に一致していた。免疫染色では，白質の希突起膠細胞はMAP 5, PHF, tau, ubiqultinに，星状膠細胞はMAP 5に陽性であった。被殼，橋核，下オリーブ核では上記の希突起膠細胞の免疫反応に加え，神経細胞はPHF, MAP5, tau, ubiqultin，星状膠細胞は被殼でtau陰性を示した以外，神経細胞と同じ免疫反応を示した。下オリーブ核，橋小脳路の神経線維はMBPでのみ陽性を示した。嗜銀性はPHF, tauと関連があることが示唆された。

A highly sensitive silver technique for glial cyto-plasmic inclusions in olivopontocerebellar atrophy ( OPCA ) was applied to 15 subjects with neuro-degenerative disorders incluing 4 patients with OPCA, 4 patients with Joseph disease and with 3 normal control subjects, and the argyrophilic struc-tures in the OPCA cases were immnuocytochemical-ly examined. As a result, the argyrophilic structures were found in the OPCA cases and Alzheimer cases. The argyrophilic structures included the white matter oligodendroglia, and neurons, astroglia and oligodendroglia in the putamen, pontine nucleus and inferior olivary nucleus. The pontocerebellar tracts and the surrounding white matter of the inferior olivary nucleus contained a small number of argyro-philic nerve fibers with a hollow structure, which were interpreted as myelin. Immunocytochemistry demonstrated that the oligodendroglia in the white matter had immunoreactivies to an paired helical filament (PHF), microtublue associated protein 5 (MAP5) , tau and ubiquitin antiserum, and the astroglia in the white matters had an immuno-reactivity to a MAP5 antiserum. In the putamen, pontine nucleus and inferior olivary nucleus, inaddition to the immunoreactivities observed in the oligodendroglia, the neurons were immunoreactive for PHF, MAP5, tau and ubiquitin antisera, and the astroglia had the same immunoreactivities as the neurons except for being tau negative in the putamen. The nerve fibers in the pontocerebellar tract and inferior olivary nucleus capsule were strongly positive for myelin basic protein and nega-tive for PHF antiserum. These findings indicate that the Gallyas positive argyrophilia in the OPCA sub-jects is closely associated with PHF or tau.