Galbraith G M, Emerson D, Fudenberg H H, Gibbs C J, Gajdusek D C
J Clin Invest. 1986 Oct;78(4):865-9. doi: 10.1172/JCI112672.
Antibodies reactive with heterologous neural tissue were detected by indirect immunofluorescence microscopy in the sera of 17 of 34 patients with retinitis pigmentosa, one of 30 normal control sera, and a variable percentage of sera derived from subjects with diverse ocular and neurological diseases. These antibodies were also found both in disease-free first degree relatives and in spouses of patients with retinitis pigmentosa. Analytical sodium dodecyl sulfate-polyacrylamide gel electrophoresis of human spinal cord components followed by immunoblots with sera under study revealed that the serum antibody was specific for the high molecular weight protein subunit of neurofilaments. No correlation was found between the presence of these antibodies and other immunological and clinical parameters in retinitis pigmentosa. These findings suggest that release of piled-up neurofilaments from damaged neurones in retinitis pigmentosa triggers B lymphocytes autoreactive to neurofilament antigens.
通过间接免疫荧光显微镜检查,在34例色素性视网膜炎患者中的17例血清、30例正常对照血清中的1例以及来自患有各种眼部和神经疾病受试者的不同比例血清中,检测到了与异源神经组织发生反应的抗体。在色素性视网膜炎患者的无病一级亲属和配偶中也发现了这些抗体。对人脊髓成分进行十二烷基硫酸钠-聚丙烯酰胺凝胶电泳分析,随后用研究中的血清进行免疫印迹,结果显示血清抗体对神经丝的高分子量蛋白亚基具有特异性。在色素性视网膜炎中,未发现这些抗体的存在与其他免疫和临床参数之间存在相关性。这些发现表明,色素性视网膜炎中受损神经元堆积的神经丝释放,触发了对神经丝抗原具有自身反应性的B淋巴细胞。