Williams L L, Shannon B T, O'Dougherty M, Wright F S
Department of Pediatrics, Children's Hospital, Columbus, OH 43205.
J Neuroimmunol. 1987 Nov;16(3):317-30. doi: 10.1016/0165-5728(87)90108-1.
Common recognized variability in the familial peripheral neuropathy, type I Charcot-Marie-Tooth disease (CMT I), led to an examination of cell-mediated immune responses in 23 CMT I patients. Increased numbers of activated T cells were found in the peripheral blood of 14 (61%) patients using fluorescent monoclonal Ta1 antibody as quantitated by flow cytometry. Altered immunoregulation was also suggested by increased levels of prostaglandin-mediated lymphocyte suppression. In the other nine CMT I patients, immune responses were normal. Lack of a relationship between Ta1 expression and CMT clinical symptoms, but with consistency within six CMT families, support the concept of immunologic heterogeneity in type I CMT with a possible genetic component.
常见的家族性周围神经病I型（夏科-马里-图斯病，CMT I）中公认的变异性，促使对23例CMT I患者的细胞介导免疫反应进行了检查。通过流式细胞术定量分析，使用荧光单克隆Ta1抗体发现14例（61%）患者外周血中活化T细胞数量增加。前列腺素介导的淋巴细胞抑制水平升高也提示免疫调节发生了改变。在其他9例CMT I患者中，免疫反应正常。Ta1表达与CMT临床症状之间缺乏相关性，但在6个CMT家族中具有一致性，这支持了I型CMT存在免疫异质性且可能有遗传成分的概念。
