Shi De-Li, Grifone Raphaëlle, Zhang Xiangmin, Li Hongyan
Laboratoire de Biologie du Développement, Sorbonne Université, CNRS UMR7622, INSERM U1156, LBD, Paris, F-75005, France.
College of Marine Life Sciences, Institute of Evolution & Marine Biodiversity, Ocean University of China, Qingdao, 266003, China.
J Muscle Res Cell Motil. 2025 Mar;46(1):53-65. doi: 10.1007/s10974-024-09685-5. Epub 2024 Nov 30.
RNA-binding proteins are critically involved in the post-transcriptional control of gene expression during embryonic development and in adult life, contributing to regulating cell differentiation and maintaining tissue homeostasis. Compared to the relatively well documented functions of transcription factors, the regulatory roles of RNA-binding proteins in muscle development and function remain largely elusive. However, deficiency of many RNA-binding proteins has been associated with muscular defects, neuromuscular disorders and heart diseases, such as myotonic dystrophy, amyotrophic lateral sclerosis, and cardiomyopathy. Rbm24 is highly conserved among vertebrates and is one of the best characterized RNA-binding proteins with crucial implication in the myogenic and cardiomyogenic programs. It presents the distinctive particularity of displaying highly restricted expression in both skeletal and cardiac muscles, with changes in subcellular localization during the process of differentiation. Functional analyses using different vertebrate models have clearly demonstrated its requirement for skeletal muscle differentiation and regeneration as well as for myocardium organization and cardiac function, by regulating the expression of both common and distinct target genes in these tissues. The challenge remains to decipher the dynamic feature of post-transcriptional circuits regulated by Rbm24 during skeletal myogenesis, cardiomyogenesis, and muscle repair. This review discusses current understanding of its function in striated muscles and its possible implication in human disease, with the aim of identifying research gaps for future investigation.
RNA结合蛋白在胚胎发育和成年期基因表达的转录后调控中起着关键作用,有助于调节细胞分化和维持组织稳态。与转录因子相对较为明确的功能相比,RNA结合蛋白在肌肉发育和功能中的调控作用仍 largely难以捉摸。然而,许多RNA结合蛋白的缺乏与肌肉缺陷、神经肌肉疾病和心脏病有关,如强直性肌营养不良、肌萎缩侧索硬化症和心肌病。Rbm24在脊椎动物中高度保守,是最具特征的RNA结合蛋白之一,对成肌和心肌生成程序具有关键影响。它具有独特的特性,即在骨骼肌和心肌中均表现出高度受限的表达,并在分化过程中发生亚细胞定位的变化。使用不同脊椎动物模型进行的功能分析清楚地表明,它通过调节这些组织中共同和独特的靶基因的表达,对骨骼肌分化和再生以及心肌组织和心脏功能是必需的。挑战仍然是破译Rbm24在骨骼肌生成、心肌生成和肌肉修复过程中调控的转录后回路的动态特征。本综述讨论了目前对其在横纹肌中的功能及其在人类疾病中可能的影响的理解,旨在确定未来研究的差距。