基因编辑通过外显子跳跃治疗杜氏肌营养不良症的临床前发展。

IF 3.4 4区 医学 Q2 CLINICAL NEUROLOGY
Journal of neuromuscular diseases Pub Date : 2025-05-01 Epub Date: 2025-03-19 DOI:10.1177/22143602251326993
Made Harumi Padmaswari, Shilpi Agrawal, Christopher E Nelson
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引用次数: 0

摘要

杜氏肌营养不良症(DMD)是由编码肌营养不良蛋白基因的功能丧失突变引起的。通过去除内部框外外显子来恢复肌营养不良蛋白的阅读框可能会解决DMD的症状。因此,在过去的二十年中,外显子跳跃的原理一直是杜氏肌营养不良症(DMD)药物开发的中心阶段。反义寡核苷酸(AONs)已被证明在调节外显子跳变的剪接位点方面是有效的,导致近年来FDA批准了几种使用该技术的药物。然而,由于AON的暂时性,研究人员正在积极探索基因组编辑作为一种潜在的长期单药治疗方法。过去十年基因组编辑技术的进步促进了这一转变。虽然在撰写本文时还没有通过基因组编辑进行DMD外显子跳跃的临床试验,但临床前研究显示了令人鼓舞的结果。这篇综述描述了基因组编辑在DMD治疗中的外显子跳变的临床前前景。除了强调基因组编辑在外显子跳变中的适应性外,本综述还描述了递送挑战并概述了未来的研究方向,这些研究方向可能为增强DMD的治疗开发奠定新的阶段。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Preclinical development of genome editing to treat Duchenne muscular dystrophy by exon skipping.

Duchenne muscular dystrophy (DMD) is caused by loss-of-function mutations to the gene encoding dystrophin. Restoring the reading frame of dystrophin by removing internal out-of-frame exons may address symptoms of DMD. Therefore, the principle of exon skipping has been at the center stage in drug development for Duchenne muscular dystrophy (DMD) over the past two decades. Antisense oligonucleotides (AONs) have proven effective in modulating splicing sites for exon skipping, resulting in the FDA approval of several drugs using this technique in recent years. However, due to the temporary nature of AON, researchers are actively exploring genome editing as a potential long-term, single-administration treatment. The advancements in genome-editing technology over the last decade have boosted this transition. While no clinical trials for exon skipping in DMD via genome editing have been conducted as of this writing, preclinical studies show encouraging results. This review describes the preclinical landscape of genome editing for exon skipping in DMD treatment. Along with highlighting the adaptability of genome editing in exon skipping, this review also describes delivery challenges and outlines future research directions that could set a new stage for enhanced therapeutic development in DMD.

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来源期刊
Journal of neuromuscular diseases
Journal of neuromuscular diseases Medicine-Neurology (clinical)
CiteScore
5.10
自引率
6.10%
发文量
102
期刊介绍: The Journal of Neuromuscular Diseases aims to facilitate progress in understanding the molecular genetics/correlates, pathogenesis, pharmacology, diagnosis and treatment of acquired and genetic neuromuscular diseases (including muscular dystrophy, myasthenia gravis, spinal muscular atrophy, neuropathies, myopathies, myotonias and myositis). The journal publishes research reports, reviews, short communications, letters-to-the-editor, and will consider research that has negative findings. The journal is dedicated to providing an open forum for original research in basic science, translational and clinical research that will improve our fundamental understanding and lead to effective treatments of neuromuscular diseases.
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