非桥粒芯糖蛋白抗体在寻常型天疱疮中的作用机制

Update of non-desmoglein IgG autoantibodies in the pathogenesis of pemphigus vulgaris

既往发现寻常型天疱疮(pemphigus vulgaris,PV)发病机制与桥粒芯糖蛋白1(desmoglein,Dsg1)和Dsg3抗体有关,目前在PV患者中发现大量非Dsg自身免疫性抗体,包括桥粒胶蛋白1(desmocollin,Dsc1)和Dsc3、斑菲素蛋白1(plakophilin,PKP1)和PKP3、斑珠蛋白(plakoglobin,PG)、桥粒斑蛋白(desmoplakin,DP)、乙酰胆碱受体等。本文对非Dsg蛋白在PV发病机制中的作用进行了综述。

The the pathogenesis of pemphigus vulgaris(PV)was associated with autoantibodies against desmosomal cadherin,desmoglein1(Dsg1)and Dsg3 in previous studies,and now many non-Dsg autoantibodies were reported including desmocollin(Dsc)1 and Dsc3,plakophilin(PKP)1 and PKP3,plakoglobin(PG),desmoplakin(DP),acetylcholine receptor(AChR),secretory pathway Ca 2+/Mn 2+-ATPase isoform 1(SPCA1),thyroid peroxidase(TPO)and mitochondrial protein.The non-Dsg autoantibodies associated with pathogenesis of PV were reviewed in this paper.