Center for Immunity and Immunotherapies, Seattle Children's Research Institute, Seattle, Washington, USA.
Mol Cell Biol. 2013 Mar;33(6):1149-63. doi: 10.1128/MCB.06407-11. Epub 2013 Jan 7.
Antigen receptors activate pathways that control cell survival, proliferation, and differentiation. Two important targets of antigen receptors, NF-κB and Jun N-terminal kinase (JNK), are activated downstream of CARMA1, a scaffolding protein that nucleates a complex including BCL10, MALT1, and other IκB kinase (IKK)-signalosome components. Somatic mutations that constitutively activate CARMA1 occur frequently in diffuse large B cell lymphoma (DLBCL) and mediate essential survival signals. Mechanisms that downregulate this pathway might thus yield important therapeutic targets. Stimulation of antigen receptors induces not only BCL10 activation but also its degradation downstream of CARMA1, thereby ultimately limiting signals to its downstream targets. Here, using lymphocyte cell models, we identify a kinase-independent requirement for TAK1 and its adaptor, TAB1, in antigen receptor-induced BCL10 degradation. We show that TAK1 acts as an adaptor for E3 ubiquitin ligases that target BCL10 for degradation. Functionally, TAK1 overexpression restrains CARMA1-dependent activation of NF-κB by reducing BCL10 levels. TAK1 also promotes counterselection of NF-κB-addicted DLBCL lines by a dual mechanism involving kinase-independent degradation of BCL10 and kinase-dependent activation of JNK. Thus, by directly promoting BCL10 degradation, TAK1 counterbalances NF-κB and JNK signals essential for the activation and survival of lymphocytes and CARMA1-addicted lymphoma types.
抗原受体激活控制细胞存活、增殖和分化的途径。CARMA1 是一种支架蛋白,它可以形成一个包含 BCL10、MALT1 和其他 IκB 激酶 (IKK) 信号小体成分的复合物,是抗原受体下游两个重要的靶点 NF-κB 和 Jun N 末端激酶 (JNK) 的激活。在弥漫性大 B 细胞淋巴瘤 (DLBCL) 中经常发生持续激活 CARMA1 的体细胞突变,介导必需的存活信号。因此,下调该途径的机制可能产生重要的治疗靶点。抗原受体的刺激不仅诱导 BCL10 的激活,而且还诱导 CARMA1 下游的降解,从而最终限制其下游靶标的信号。在这里,我们使用淋巴细胞细胞模型,确定了 TAK1 和其衔接子 TAB1 在抗原受体诱导的 BCL10 降解中不需要激酶的作用。我们表明,TAK1 作为 E3 泛素连接酶的衔接子,将 BCL10 作为降解的靶标。功能上,TAK1 通过降低 BCL10 水平来限制 CARMA1 依赖性 NF-κB 的激活。TAK1 还通过涉及 BCL10 激酶独立降解和 JNK 激酶依赖性激活的双重机制,促进 NF-κB 成瘾的 DLBCL 细胞系的反向选择。因此,通过直接促进 BCL10 降解,TAK1 平衡了 NF-κB 和 JNK 信号,这些信号对于淋巴细胞和 CARMA1 成瘾型淋巴瘤类型的激活和存活至关重要。