The Kennedy Institute of Rheumatology, University of Oxford, Oxford OX3 7FY, UK.
Int J Mol Sci. 2024 Jul 14;25(14):7705. doi: 10.3390/ijms25147705.
The Ro60/SSA2 autoantigen is an RNA-binding protein and a core component of nucleocytoplasmic ribonucleoprotein (RNP) complexes. Ro60 is essential in RNA metabolism, cell stress response pathways, and cellular homeostasis. It stabilises and mediates the quality control and cellular distribution of small RNAs, including YRNAs (for the 'y' in 'cytoplasmic'), retroelement transcripts, and misfolded RNAs. Ro60 transcriptional dysregulation or loss of function can result in the generation and release of RNA fragments from YRNAs and other small RNAs. Small RNA fragments can instigate an inflammatory cascade through endosomal toll-like receptors (TLRs) and cytoplasmic RNA sensors, which typically sense pathogen-associated molecular patterns, and mount the first line of defence against invading pathogens. However, the recognition of host-originating RNA moieties from Ro60 RNP complexes can activate inflammatory response pathways and compromise self-tolerance. Autoreactive B cells may produce antibodies targeting extracellular Ro60 RNP complexes. Ro60 autoantibodies serve as diagnostic markers for various autoimmune diseases, including Sjögren's disease (SjD) and systemic lupus erythematosus (SLE), and they may also act as predictive markers for anti-drug antibody responses among rheumatic patients. Understanding Ro60's structure, function, and role in self-tolerance can enhance our understanding of the underlying molecular mechanisms of autoimmune conditions.
Ro60/SSA2 自身抗原是一种 RNA 结合蛋白,也是核细胞质核糖核蛋白 (RNP) 复合物的核心组成部分。Ro60 在 RNA 代谢、细胞应激反应途径和细胞内稳态中起着至关重要的作用。它稳定并介导小分子 RNA(包括细胞质 YRNA、逆转录元件转录本和错误折叠 RNA)的质量控制和细胞分布。Ro60 的转录失调或功能丧失可导致 YRNA 和其他小分子 RNA 产生和释放 RNA 片段。小 RNA 片段可通过内体 Toll 样受体 (TLR) 和细胞质 RNA 传感器引发炎症级联反应,这些传感器通常识别病原体相关的分子模式,并对入侵病原体发起第一道防线。然而,来自 Ro60 RNP 复合物的宿主来源 RNA 片段的识别可激活炎症反应途径并损害自身耐受性。自身反应性 B 细胞可能产生针对细胞外 Ro60 RNP 复合物的抗体。Ro60 自身抗体可作为各种自身免疫性疾病的诊断标志物,包括干燥综合征 (SjD) 和系统性红斑狼疮 (SLE),它们也可能作为风湿性疾病患者抗药物抗体反应的预测标志物。了解 Ro60 的结构、功能及其在自身耐受性中的作用可以增强我们对自身免疫性疾病潜在分子机制的理解。