Bugiani O, Giaccone G, Verga L, Pollo B, Frangione B, Farlow M R, Tagliavini F, Ghetti B
Istituto Neurologico Carlo Besta, Milan, Italy.
J Neuropathol Exp Neurol. 1993 Jan;52(1):64-70. doi: 10.1097/00005072-199301000-00008.
Gerstmann-Sträussler-Scheinker disease in the Indiana kindred is pathologically characterized by deposits of PrP-amyloid, neurofibrillary tangles and degenerating neurites. The aim of this study was to investigate seven patients of different ages for beta PP and A beta immunoreactivities associated with PrP-amyloid deposits and degenerating neurites. In one asymptomatic individual with PrP-amyloid deposits, Alz50 and A beta immunoreactivities were absent. In six symptomatic patients, the degenerating neurites surrounding PrP-amyloid deposits were labeled by Alz50 and by antibodies to synaptophysin, ubiquitin and the N- and C-terminal domains of beta PP. In one symptomatic, senile patient, A beta immunoreactivity was present in the extracellular space, often in association with PrP-amyloid deposits. The analysis of the immunohistochemical findings suggested that in the Indiana kindred the intracellular accumulation of beta PP, synaptophysin and ubiquitinated material most probably revealed a reaction of neurites to PrP-amyloid, whereas the extracellular deposition of A beta was likely an age-related phenomenon.
印第安纳家族性格斯特曼-施特劳斯勒-申克病的病理特征是朊蛋白淀粉样沉积、神经原纤维缠结和神经突退变。本研究的目的是调查7名不同年龄患者与朊蛋白淀粉样沉积和神经突退变相关的β淀粉样前体蛋白（beta PP）和β淀粉样蛋白（Aβ）免疫反应性。在1例有朊蛋白淀粉样沉积的无症状个体中，未检测到Alz50和Aβ免疫反应性。在6例有症状的患者中，围绕朊蛋白淀粉样沉积的退变神经突被Alz50以及抗突触素、泛素和β淀粉样前体蛋白N端和C端结构域的抗体标记。在1例有症状的老年患者中，细胞外间隙存在Aβ免疫反应性，且常与朊蛋白淀粉样沉积相关。免疫组化结果分析表明，在印第安纳家族中，β淀粉样前体蛋白、突触素和泛素化物质的细胞内积聚很可能揭示了神经突对朊蛋白淀粉样沉积的反应，而Aβ的细胞外沉积可能是一种与年龄相关的现象。
