Pelacho B, Natal C, España A, Sánchez-Carpintero I, Iraburu M J, López-Zabalza M J
Department of Biochemistry, University of Navarra, Apartado 177, 31080 Pamplona, Spain.
FEBS Lett. 2004 May 21;566(1-3):6-10. doi: 10.1016/j.febslet.2004.03.107.
Pemphigus vulgaris (PV) is an autoimmune disease characterized by binding of IgG autoantibodies to epidermal keratinocyte desmosomes. IgG autoantibodies obtained from a patient with mucocutaneous PV reacted with plakoglobin (Plkg) in addition to desmoglein-3 (Dsg3) and Dsg1. Immunofluorescence analysis confirmed that IgG autoantibodies, unlike antibodies from a healthy volunteer, caused disruption of cell-cell contacts in HaCaT keratinocytes. Moreover, apoptosis was enhanced in cells treated with autoantibodies compared to those treated with normal antibodies. The apoptotic process induced by IgG autoantibodies was characterized by caspase-3 activation, Bcl-2 depletion and Bax expression. The present report demonstrates that PV IgG autoantibodies promote apoptosis in HaCaT keratinocytes.
寻常型天疱疮(PV)是一种自身免疫性疾病,其特征是IgG自身抗体与表皮角质形成细胞桥粒结合。从一位患有黏膜皮肤型PV的患者身上获取的IgG自身抗体,除了与桥粒芯糖蛋白3(Dsg3)和桥粒芯糖蛋白1(Dsg1)发生反应外,还与桥粒斑珠蛋白(Plkg)发生反应。免疫荧光分析证实,与来自健康志愿者的抗体不同,IgG自身抗体导致了HaCaT角质形成细胞中细胞间接触的破坏。此外,与用正常抗体处理的细胞相比,用自身抗体处理的细胞中凋亡增强。由IgG自身抗体诱导的凋亡过程的特征是半胱天冬酶-3激活、Bcl-2耗竭和Bax表达。本报告表明,PV IgG自身抗体可促进HaCaT角质形成细胞的凋亡。