Walls R S, Ruwoldt A
J Clin Invest. 1984 Jul;74(1):165-72. doi: 10.1172/JCI111397.
The hypothesis that an immune response is directed to components of brain tissue in Huntington's disease (HD) was examined. 27 patients with HD, 21 children of affected parents who showed no signs of disease, 6 spouses of HD patients, and 40 patients with other neurologic diseases were investigated. Leukocyte migration inhibition to antigens prepared from brain tissue was demonstrated in 26 HD patients and in 9 children. None of the six spouses reacted. 13 patients with other neurologic conditions (including 60% of patients after cerebrovascular accidents) reacted to rat brain extract. Fractionation of brain tissue indicated that the material responsible for reactions in these patients differed from that in HD. The material that elicited hypersensitivity in HD was not species specific, since it was found in rat, calf, human, and rabbit brain. It was not present in rat kidney, liver, or heart. Preliminary characterization showed that the material was protein in nature, contained sugar and lipid, and was heat labile. The finding of hypersensitivity in about half the unaffected children of HD parents suggests that it may precede the clinical onset of disease and serve as a marker for the gene.
对亨廷顿舞蹈症（HD）中免疫反应针对脑组织成分这一假说进行了检验。研究了27例HD患者、21名患病父母的子女（未表现出疾病迹象）、6名HD患者的配偶以及40例患有其他神经系统疾病的患者。在26例HD患者和9名儿童中证实了白细胞对脑组织制备抗原的迁移抑制。6名配偶均无反应。13例患有其他神经系统疾病的患者（包括60%的脑血管意外后患者）对大鼠脑提取物有反应。脑组织分级分离表明，引起这些患者反应的物质与HD患者的不同。在HD中引起超敏反应的物质不是种属特异性的，因为在大鼠、小牛、人类和兔脑中均有发现。大鼠肾脏、肝脏或心脏中不存在该物质。初步鉴定表明该物质本质上是蛋白质，含有糖和脂质，且对热不稳定。在HD父母约一半未患病的子女中发现超敏反应，这表明超敏反应可能在疾病临床发作之前出现，并可作为该基因的一个标志物。
