Department of Biological Chemistry, Institute for Cell Engineering, The Johns Hopkins University School of Medicine, Baltimore, MD, United States.
Front Immunol. 2018 Sep 19;9:2105. doi: 10.3389/fimmu.2018.02105. eCollection 2018.
CARD11 functions as a key signaling scaffold that controls antigen-induced lymphocyte activation during the adaptive immune response. Somatic mutations in CARD11 are frequently found in Non-Hodgkin lymphoma, and at least three classes of germline CARD11 mutations have been described as the basis for primary immunodeficiency. In this review, we summarize our current understanding of how CARD11 signals, how its activity is regulated, and how mutations bypass normal regulation to cause disease.
CARD11 作为一个关键的信号支架，在适应性免疫反应期间控制抗原诱导的淋巴细胞激活。CARD11 的体细胞突变经常在非霍奇金淋巴瘤中发现，至少有三类种系 CARD11 突变被描述为原发性免疫缺陷的基础。在这篇综述中，我们总结了我们目前对 CARD11 信号转导、其活性如何调节以及突变如何绕过正常调节导致疾病的理解。
