桥粒胶蛋白3
天疱疮
医学
大疱性类天疱疮
类天疱疮
自身免疫性疾病
疾病
免疫学
抗体
自身抗体
病理
作者
Christoph T. Ellebrecht,Damián Maseda,Aimee Payne
标识
DOI:10.1016/j.jid.2021.04.040
摘要
Pemphigus and pemphigoid are paradigms for understanding the mechanisms of antibody-mediated autoimmune disease in humans. In pemphigus, IgG4-predominant autoantibodies cause intraepidermal blistering by direct interference with desmoglein interactions and subsequent disruption of desmosomes and signaling pathways. In pemphigoid, IgG1, IgG4, and IgE autoantibodies against basement membrane zone antigens directly interfere with hemidesmosomal adhesion, activating complement and Fc receptor‒mediated effector pathways. Unraveling disease mechanisms in pemphigus and pemphigoid has identified numerous opportunities for clinical trials, which hold promise to identify safer and more effective therapies for these potentially life-threatening diseases.
科研通智能强力驱动
Strongly Powered by AbleSci AI