Giaccone G, Tagliavini F, Street J S, Ghetti B, Bugiani O
Istituto Neurologico Carlo Besta, Milano, Italy.
Acta Neuropathol. 1988;77(1):14-20. doi: 10.1007/BF00688237.
In a patient with progressive supranuclear palsy (PSP) and hypertrophy of the olives, neurons with different forms of argyrophilic degeneration were detected by means of Bodian's silver staining method, i.e., neurofibrillary tangle-bearing neurons in the basal ganglia and brain stem, ballooned argyrophilic neurons in the brain stem, and hypertrophied neurons in the olives. In these cells, the cytoskeleton was investigated to ascertain whether neurons with different cytoskeletal changes contained phosphorylated neurofilaments (P-Nf) in the perikaryon. This study, carried out using two monoclonal antibodies that recognize phosphorylated epitopes of the neurofilament high molecular weight subunits, showed that hypertrophied olivary neurons, most ballooned neurons and a small aliquot of tangle-bearing neurons were labelled. The immunostaining of hypertrophied and ballooned neurons was localized in the whole perikaryon and dendrites, whereas that of tangle-bearing neurons was confined to the tangle. These findings were reproduced in five additional patients (one with hypertrophy of the olives, four with PSP) and demonstrated that, in PSP, the mechanism responsible for tangle formation does not affect the ability of neurons to accumulate P-Nf. This fact suggested that perikaryonal P-Nf accumulation is likely to be part of the cell reaction to abnormal conditions affecting the neuronal cytoskeleton.
在一名患有进行性核上性麻痹（PSP）且橄榄体肥大的患者中，通过博迪安银染色法检测到具有不同形式嗜银性退变的神经元，即基底神经节和脑干中带有神经原纤维缠结的神经元、脑干中气球样嗜银神经元以及橄榄体中的肥大神经元。在这些细胞中，对细胞骨架进行了研究，以确定具有不同细胞骨架变化的神经元在核周体中是否含有磷酸化神经丝（P-Nf）。本研究使用两种识别神经丝高分子量亚基磷酸化表位的单克隆抗体进行，结果显示肥大的橄榄体神经元、大多数气球样神经元以及一小部分带有缠结的神经元被标记。肥大和气球样神经元的免疫染色定位于整个核周体和树突，而带有缠结的神经元的免疫染色局限于缠结部位。另外5名患者（1名橄榄体肥大，4名PSP）也重现了这些发现，表明在PSP中，负责缠结形成的机制并不影响神经元积累P-Nf的能力。这一事实表明，核周体P-Nf积累可能是细胞对影响神经元细胞骨架的异常状况作出反应的一部分。
