Amyloids are implicated in neurodegenerative and systemic diseases, yet they serve important functional roles in numerous organisms. Heterogeneous nuclear ribonucleoproteins (hnRNPs) represent a large family of RNA-binding proteins (RBPs) that control central events of RNA biogenesis in normal and diseased cellular conditions. Many of these proteins contain prion-like sequences of low complexity, which not only assemble into functional fibrils in response to cellular cues but can also lead to disease when missense mutations arise in their sequences. Recent advances in cryo-electron microscopy (cryo-EM) have provided unprecedented high-resolution structural insights into diverse amyloid assemblies formed by hnRNPs and structurally related RBPs, including TAR DNA-binding protein 43 (TDP-43), Fused in Sarcoma (FUS), Orb2, hnRNPA1, hnRNPA2, and hnRNPDL-2. This review provides a comprehensive overview of these structures and explores their functional and pathological implications.

淀粉样蛋白与神经退行性疾病和系统性疾病有关，但它们在许多生物体中发挥着重要的功能作用。异质核核糖核蛋白 (hnRNP) 代表了一个大家族的 RNA 结合蛋白 (RBP)，其控制正常和患病细胞条件下 RNA 生物发生的中心事件。许多这些蛋白质含有低复杂性的类朊病毒序列，这些序列不仅响应细胞信号组装成功能性原纤维，而且当其序列中出现错义突变时也可能导致疾病。冷冻电子显微镜 (cryo-EM) 的最新进展为 hnRNP 和结构相关的 RBP 形成的多种淀粉样蛋白组装体提供了前所未有的高分辨率结构见解，包括 TAR DNA 结合蛋白 43 (TDP-43)、肉瘤融合蛋白 (FUS) )、Orb2、hnRNPA1、hnRNPA2 和 hnRNPDL-2。本综述对这些结构进行了全面的概述，并探讨了它们的功能和病理学意义。