核内不均一核糖核蛋白属于RNA结合蛋白中的一类，具有高度保守的结构。其中，核内不均一核糖核蛋白A2/B1(hnRNPA2/B1)参与转录、翻译等多种过程。近年来，神经退行性疾病研究领域发现，hnRNPA2/B1促进细胞质内纤维蛋白聚集，参与神经元内应激颗粒形成，调节多种认知功能相关过程，与肌萎缩侧索硬化、额颞叶痴呆、阿尔茨海默病等神经退行性疾病存在密切联系。本文综述相关研究，对hnRNPA2/B1在神经退行性疾病中的重要作用与机制进行总结，为未来神经退行性疾病诊断及治疗提供帮助。“,”Heterogeneous nuclear rib nucleoproteins belong to a class of RNA-binding proteins and possess highly conserved structures. Among them, the heterogeneous nuclear rib nucleoprotein A2/B1 (heterogeneous nuclear rib nucleoprotein A2, hnRNPA2/B1) is involved in many processes such as transcription and translation. In recent years, in the field of neurodegenerative diseases, it has been found that hnRNPA2/B1 promotes the accumulation of fibrin in the cytoplasm and participates in the formation of stress granules in neurons, which involves a variety of processes related to cognitive function, and is closely related to neurodegenerative diseases such as amyotrophic lateral sclerosis, frontotemporal dementia, Alzheimer′s disease and so on. This article reviews the related research, summarizes the important role and mechanism of hnRNPA2/B1 in neurodegenerative diseases, and provides help for the diagnosis and treatment of neurodegenerative diseases in the future.