DEAD-box 解旋酶是参与细胞 RNA 代谢的许多方面的多功能蛋白质。 DEAD-box 解旋酶 41 (DDX41) 在先天免疫感应和造血稳态中发挥着关键作用。 DDX41 识别微生物感染过程中产生的外源或自身核酸，从而启动抗病原体反应。 DDX41 还与 RNA (R) 环结合，该结构由 DNA/RNA 杂合体和转录过程中发生的 DNA 置换链组成，从而通过防止其积累来维持基因组稳定性。 DDX41 缺乏会导致 R 环水平升高，从而导致炎症反应，可能影响造血干细胞和祖细胞的产生和发育。除了核酸结合之外，DDX41 还与参与 RNA 剪接的蛋白质以及参与先天免疫的细胞蛋白质结合。 DDX41 也是家族性和散发性骨髓增生异常综合征/急性髓性白血病 (MDS/AML) 的肿瘤抑制因子。在本综述中，我们总结了 DDX 解旋酶在关键生物过程中的功能，特别关注 DDX41 与细胞分子的关联及其在先天免疫、造血和骨髓恶性肿瘤发展中的作用机制。版权所有 © 2024 Ma 和 Ross。

DEAD-box helicases are multifunctional proteins participating in many aspects of cellular RNA metabolism. DEAD-box helicase 41 (DDX41) in particular has pivotal roles in innate immune sensing and hematopoietic homeostasis. DDX41 recognizes foreign or self-nucleic acids generated during microbial infection, thereby initiating anti-pathogen responses. DDX41 also binds to RNA (R)-loops, structures consisting of DNA/RNA hybrids and a displaced strand of DNA that occur during transcription, thereby maintaining genome stability by preventing their accumulation. DDX41 deficiency leads to increased R-loop levels, resulting in inflammatory responses that likely influence hematopoietic stem and progenitor cell production and development. Beyond nucleic acid binding, DDX41 associates with proteins involved in RNA splicing as well as cellular proteins involved in innate immunity. DDX41 is also a tumor suppressor in familial and sporadic myelodysplastic syndrome/acute myelogenous leukemia (MDS/AML). In the present review, we summarize the functions of DDX helicases in critical biological processes, particularly focusing on DDX41's association with cellular molecules and the mechanisms underlying its roles in innate immunity, hematopoiesis and the development of myeloid malignancies.Copyright © 2024 Ma and Ross.