Presence of autoimmune regulator and absence of desmoglein 1 in thymoma associated with a pemphigus foliaceus patient.
Presence of autoimmune regulator and absence of desmoglein 1 in thymoma associated with a pemphigus foliaceus patient.
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落叶型天疱疮患者相关胸腺瘤中存在自身免疫调节剂且桥粒芯糖蛋白 1 缺失。
DOI：
10.1111/bjd.13617
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发表时间：
2015
期刊：
影响因子：
--
通讯作者：
A.
中科院分区：
文献类型：
--
作者：
Tsuchisaka;A.
亲爱的编辑，桥粒芯糖蛋白（Dsg）1和Dsg 3分别是落叶型天疱疮（PF）和寻常型天疱疮（PV）的自身抗原。1，2胸腺瘤经常与各种自身免疫性疾病相关，特别是重症肌无力（MG）。3胸腺瘤在组织病理学上分为A、AB、B1、B2、B3和C型（更名为胸腺癌）。4自身免疫调节因子（AIRE）是自身免疫性多内分泌病-念珠菌病-外胚层营养不良（APECED）的致病分子，在胎儿胸腺髓质表达。5，6 AIRE通过表达组织特异性抗原（TSA）调节自身反应性T细胞的阴性选择。因此，AIRE参与中枢免疫耐受和自身免疫性疾病的发展。7 AIRE也在非胸腺组织中表达，包括淋巴结和脾脏，但在所有胸腺瘤中均不表达，除了少数B1型胸腺瘤。8，9 b5 t是胸腺特异性蛋白酶体S28的一个亚单位，参与胸腺中抗原的加工，在B型胸腺瘤中表达，而在A型胸腺瘤中不表达。十，十一
DEAR EDITOR, Desmoglein (Dsg) 1 and Dsg3 are autoantigens for pemphigus foliaceus (PF) and pemphigus vulgaris (PV), respectively. 1, 2 Thymomas are frequently associated with various autoimmune diseases, particularly myasthenia gravis (MG). 3 Thymomas are divided histopathologically into types A, AB, B1, B2, B3 and C (renamed thymic carcinoma). 4 Autoimmune regulator (AIRE) is a causative molecule in autoimmune polyendocrinopathy–candidiasis–ectodermal dystrophy (APECED), and is expressed in thymic medulla in fetal thymus. 5, 6 AIRE regulates negative selection of self-reactive T cells by expressing tissue-specific antigens (TSAs). Thus, AIRE is involved in central immune tolerance and the development of autoimmune diseases. 7 AIRE is also expressed in nonthymus tissues, including lymph nodes and spleen, but is absent in all thymomas, except for a few type B1 thymomas. 8, 9 b5t, a subunit of thymus-specific proteasome S28, is involved in antigen processing for positive selection in thymus, and is expressed in type B, but not type A, thymomas. 10, 11