Loeser Stefanie, Penninger Josef M
IMBA, Institute of Molecular Biotechnology of the Austrian Academy of Sciences, Dr. Bohrgasse 3, A-1030 Vienna, Austria.
Semin Immunol. 2007 Jun;19(3):206-14. doi: 10.1016/j.smim.2007.02.004. Epub 2007 Mar 27.
The family of the Casitas B-lineage Lymphoma (Cbl) proteins, c-Cbl, Cbl-b, and Cbl-3, function as E3 ubiquitin ligases and molecular adaptors. In particular, Cbl-b acts as a gatekeeper in T cell activation that controls activation thresholds and the requirement for co-stimulation. Loss of Cbl-b expression renders animals susceptible to antigen-triggered autoimmunity suggesting that Cbl-b is a key autoimmunity gene. In addition, Cbl-b plays a critical role in T cell anergy and escape from regulatory T cells (Treg) suppression. Modulation of Cbl-b might provide us with a unique opportunity for future immune treatment of human disorders such as autoimmunity, immunodeficiency, or cancer.
Casitas B系淋巴瘤(Cbl)蛋白家族,即c-Cbl、Cbl-b和Cbl-3,作为E3泛素连接酶和分子衔接子发挥作用。特别是,Cbl-b在T细胞活化过程中充当把关者,控制活化阈值和共刺激需求。Cbl-b表达缺失使动物易患抗原触发的自身免疫,这表明Cbl-b是一个关键的自身免疫基因。此外,Cbl-b在T细胞无能以及逃脱调节性T细胞(Treg)抑制中起关键作用。调节Cbl-b可能为我们未来免疫治疗人类疾病(如自身免疫、免疫缺陷或癌症)提供独特的机会。