Texas Children's Cancer Center, Department of Pediatrics, 2 Department of Pathology and Immunology, Dan L. Duncan Cancer Center, Baylor College of Medicine, Houston, TX 77030.
J Exp Med. 2014 Feb 10;211(2):313-28. doi: 10.1084/jem.20122844. Epub 2014 Feb 3.
Lys63-linked polyubiquitination of RIG-I is essential in antiviral immune defense, yet the molecular mechanism that negatively regulates this critical step is poorly understood. Here, we report that USP21 acts as a novel negative regulator in antiviral responses through its ability to bind to and deubiquitinate RIG-I. Overexpression of USP21 inhibited RNA virus-induced RIG-I polyubiquitination and RIG-I-mediated interferon (IFN) signaling, whereas deletion of USP21 resulted in elevated RIG-I polyubiquitination, IRF3 phosphorylation, IFN-α/β production, and antiviral responses in MEFs in response to RNA virus infection. USP21 also restricted antiviral responses in peritoneal macrophages (PMs) and bone marrow-derived dendritic cells (BMDCs). USP21-deficient mice spontaneously developed splenomegaly and were more resistant to VSV infection with elevated production of IFNs. Chimeric mice with USP21-deficient hematopoietic cells developed virus-induced splenomegaly and were more resistant to VSV infection. Functional comparison of three deubiquitinases (USP21, A20, and CYLD) demonstrated that USP21 acts as a bona fide RIG-I deubiquitinase to down-regulate antiviral response independent of the A20 ubiquitin-editing complex. Our studies identify a previously unrecognized role for USP21 in the negative regulation of antiviral response through deubiquitinating RIG-I.
Lys63 连接的 RIG-I 多泛素化对于抗病毒免疫防御至关重要,但负调控这一关键步骤的分子机制仍知之甚少。在这里,我们报告 USP21 通过与 RIG-I 结合并去泛素化来充当抗病毒反应中的新型负调控因子。USP21 的过表达抑制了 RNA 病毒诱导的 RIG-I 多泛素化和 RIG-I 介导的干扰素 (IFN) 信号转导,而 USP21 的缺失导致 MEFs 中 RIG-I 多泛素化、IRF3 磷酸化、IFN-α/β产生和抗病毒反应增加,从而响应 RNA 病毒感染。USP21 还限制了腹膜巨噬细胞 (PMs) 和骨髓来源的树突状细胞 (BMDCs) 中的抗病毒反应。USP21 缺陷小鼠自发发展为脾肿大,并对 VSV 感染具有更高的抵抗力,IFN 产生增加。具有 USP21 缺陷造血细胞的嵌合小鼠发展出病毒诱导的脾肿大,并对 VSV 感染具有更高的抵抗力。三种去泛素酶(USP21、A20 和 CYLD)的功能比较表明,USP21 作为一种真正的 RIG-I 去泛素酶,通过去泛素化 RIG-I 来独立于 A20 泛素编辑复合物下调抗病毒反应。我们的研究确定了 USP21 在负调控抗病毒反应中的先前未被认识的作用,通过去泛素化 RIG-I。