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患有 AIRE 缺陷的患者具有独特的高 afinity 疾病改善的自身抗体
Steffen Meyer1, Martin Woodward2, Christina Hertel1
1ImmunoQure AG, Königsallee 90, 2012 Düsseldorf, Germany.
Cell
|July 19, 2016
概括
在APS1/APECED患者中,自身免疫调节器 (AIRE) 缺陷会降低B细胞耐受性,导致自身反应性抗体. 高亲和度的自身抗体,尤其是针对I型干扰素的抗体,可能会限制疾病的进展.
科学领域:
- 免疫学
- 自体免疫性
- 分子生物学
背景情况:
- 自体免疫多胞体病变 - 淋巴瘤 - 皮肤外膜发育不良综合征 (APECED) 是由自身免疫调节基因 (AIRE) 的缺陷引起的.
- AIRE对于中央T细胞耐受性至关重要,但其在B细胞耐受性中的作用不明.
- 缺乏AIRE的个体的B细胞耐受性缺陷可能导致自身反应性抗体的产生.
研究的目的:
- 研究APS1/APECED患者B细胞自身反应的程度和性质.
- 确定在抗体成熟过程中B细胞耐受性丧失的阶段.
- 评估自然存在的APS1/APECED自身抗体的生物活性和潜在的治疗效用.
主要方法:
- 对81名APS1/APECED患者的B细胞自身反应的分析.
- 鉴定自身抗体亲和力和中和能力,特别是针对细胞因子.
- 在体外和体内对自身抗体生物活性进行评估,包括I型干扰素中和.
主要成果:
- 大多数APS1/APECED患者表现出B细胞对不同类型的自我蛋白的自身反应,自身抗体检测出数千种人体蛋白.
- 在依赖T细胞的抗体亲和力成熟过程中,似乎发生了B细胞耐受性的损失.
- 高亲和性,中和性自身抗体,特别是针对I型干扰素,是普遍存在的,并且具有生物活性.
- 中和I型干扰素自身抗体与I型糖尿病发生率呈反向相关性.
结论:
- 在APS1/APECED患者中,AIRE缺乏导致显著的B细胞自身反应和自身抗体产生.
- 自身抗体,特别是那些能中和I型干扰素的抗体,可能对某些自身免疫性疾病 (如I型糖尿病) 起到保护作用.
- 在APS1/APECED患者中自然存在的自身抗体有可能用于治疗自身免疫疾病.
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