Martin J E, Mather K, Swash M, Dodd S M, Dale G E, Garofalo O, Leigh P N
Institute of Pathology, London Hospital.
Neuropathol Appl Neurobiol. 1991 Apr;17(2):105-11. doi: 10.1111/j.1365-2990.1991.tb00701.x.
Dialysis encephalopathy, a complication of long-term haemodialysis, is a syndrome characterized by progressive dementia, myoclonus, dysarthria and ataxia associated with high serum and brain levels of aluminium. Expression of heat-shock or stress proteins, including ubiquitin can be induced in cell culture experiments by aluminium. We report immunohistochemical studies of heat shock protein (HSP) expression in the frontal cortex of three patients with dialysis dementia. Immunolabelling with antibody to the 72 kD heat shock protein revealed punctate granules in most endothelial cells of cortical vessels in patients with dialysis encephalopathy. These granules, 1-5 microns in diameter, aggregated to form inclusions that resembled stress-granules, typically induced in plant or animal cell culture by repeated insult. These granules did not express epitopes of ubiquitin. They were rare in endothelial cells in the brains of subjects dying with other neurological disorders or of non-neurological causes. We suggest that these stress granules represent a toxic response of endothelial cells in the brain to aluminium.
透析性脑病是长期血液透析的一种并发症,是一种以进行性痴呆、肌阵挛、构音障碍和共济失调为特征的综合征,与血清和脑内铝含量升高有关。在细胞培养实验中,铝可诱导包括泛素在内的热休克蛋白或应激蛋白的表达。我们报告了对三名透析性痴呆患者额叶皮质中热休克蛋白(HSP)表达的免疫组织化学研究。用抗72kD热休克蛋白抗体进行免疫标记显示,透析性脑病患者皮质血管的大多数内皮细胞中有点状颗粒。这些颗粒直径为1-5微米,聚集形成类似应激颗粒的包涵体,通常在植物或动物细胞培养中由反复损伤诱导产生。这些颗粒不表达泛素表位。在死于其他神经系统疾病或非神经系统原因的受试者大脑的内皮细胞中很少见。我们认为这些应激颗粒代表了大脑内皮细胞对铝的毒性反应。