抗色氨酸羟化酶-1 和抗 AIE-75 自身抗体在 APECED 和 IPEX 综合征中的疾病特异性。
Disease specificity of anti-tryptophan hydroxylase-1 and anti-AIE-75 autoantibodies in APECED and IPEX syndrome.
机构信息
Department of Pediatrics, Hokkaido University Graduate School of Medicine, Sapporo, Japan; Department of Dentistry for Children and Disabled Persons, Hokkaido University Graduate School of Dental Medicine, Sapporo, Japan.
Department of Pediatrics, Hokkaido University Graduate School of Medicine, Sapporo, Japan.
出版信息
Clin Immunol. 2015 Jan;156(1):36-42. doi: 10.1016/j.clim.2014.10.010. Epub 2014 Nov 8.
Autoantibodies to autoimmune enteropathy-related 75 kDa antigen (AIE-75) and villin are disease markers of immune dysregulation, polyendocrinopathy, enteropathy, X-linked (IPEX) syndrome which is characterized by a peripheral tolerance defect. On the other hand, anti-tryptophan hydroxylase-1 (TPH-1) antibodies are detected in autoimmune polyendocrinopathy, candidiasis, ectodermal dystrophy (APECED), a central tolerance defect, especially when complicated with gastrointestinal dysfunction. However, to date, anti-AIE-75 and anti-villin antibodies or anti-TPH-1 antibodies have not been tested in APECED or IPEX syndrome, respectively. In the present study, we confirmed the disease specificity of both anti-AIE-75 and anti-TPH-1, although anti-villin antibodies were detected in some patients with APECED. Our observation suggests that immunotolerance to AIE-75 depends on the peripheral mechanism, whereas the tolerance to TPH-1 depends on the central mechanisms.
自身免疫性肠炎相关 75kDa 抗原（AIE-75）和微管蛋白自身抗体是免疫失调、多内分泌腺病、肠病、X 连锁（IPEX）综合征的疾病标志物，其特征为外周耐受缺陷。另一方面，抗色氨酸羟化酶-1（TPH-1）抗体见于自身免疫性多内分泌腺病、念珠菌病、外胚层营养不良（APECED），为中枢耐受缺陷，尤其是伴有胃肠功能障碍时。然而，迄今为止，尚未分别在 APECED 或 IPEX 综合征中检测到抗 AIE-75 和抗 villin 抗体或抗 TPH-1 抗体。在本研究中，我们证实了抗 AIE-75 和抗 TPH-1 的疾病特异性，尽管在一些 APECED 患者中检测到抗 villin 抗体。我们的观察表明，AIE-75 的免疫耐受依赖于外周机制，而 TPH-1 的耐受依赖于中枢机制。
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