杜氏肌营养不良的细胞治疗:希望、挑战和争议。

IF 6.2 2区 生物学 Q1 BIOCHEMISTRY & MOLECULAR BIOLOGY
Agnieszka Łoboda, Józef Dulak
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引用次数: 0

摘要

尽管进行了广泛的研究,但杜氏肌营养不良症(Duchenne muscular dystrophy)仍然是一种无法治愈的疾病。杜氏肌营养不良症是一种由缺乏肌营养不良蛋白(一种关键的肌肉结构蛋白)引起的神经肌肉疾病。目前正在研究的潜在治疗方案之一是细胞治疗,尽管它尚未在临床上建立。几种策略,包括肌肉卫星细胞、成血管细胞(血管相关多能干细胞)和诱导多能干细胞(iPSC)衍生的肌肉细胞,已经成为恢复肌营养不良蛋白表达和再生受损肌肉组织的工具。然而,每一种方法都面临着显著的局限性,包括细胞植入不良、传递效率低和免疫排斥的风险。此外,必须严格评估长期安全性、致瘤性可能性和脱靶效应。重要的是,后一种技术利用从iPSC分化的心肌细胞,具有解决心肌病的潜力,心肌病是DMD患者死亡的主要原因。与此同时,一些声称具有干细胞潜力的细胞的干预已经出现,这引起了科学和伦理方面的关注。这篇综述总结了DMD细胞疗法的最新进展,突出了有希望的进展,同时批判性地分析了有问题的方法。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Cell therapy for Duchenne muscular dystrophy: promises, challenges, and controversies.

Despite extensive studies, Duchenne muscular dystrophy, a neuromuscular disorder caused by the lack of dystrophin, a key muscle structural protein, remains an incurable disease. One of the potential treatment options currently being investigated is cell therapy, although it has not yet been clinically established. Several strategies, including muscle satellite cells, mesoangioblasts (vessel-associated multipotent stem cells), and induced pluripotent stem cell (iPSC)-derived muscle cells, have emerged as tools for restoring dystrophin expression and regenerating damaged muscle tissue. Nevertheless, each of these approaches faces significant limitations, including poor cell engraftment, low delivery efficiency, and the risk of immune rejection. Furthermore, long-term safety, the possibility of tumorigenicity, and off-target effects must be rigorously evaluated. Importantly, the latter technology, utilizing cardiomyocytes differentiated from iPSC, holds the potential for addressing cardiomyopathy, the major cause of death of DMD patients. At the same time, several interventions using cells with claimed stem cell potential have emerged, raising both scientific and ethical concerns. This review summarizes recent advancements in the development of cell therapies for DMD, highlighting promising progress while critically analysing questionable approaches.

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来源期刊
Cellular and Molecular Life Sciences
Cellular and Molecular Life Sciences 生物-生化与分子生物学
CiteScore
13.20
自引率
1.20%
发文量
546
审稿时长
1.0 months
期刊介绍: Journal Name: Cellular and Molecular Life Sciences (CMLS) Location: Basel, Switzerland Focus: Multidisciplinary journal Publishes research articles, reviews, multi-author reviews, and visions & reflections articles Coverage: Latest aspects of biological and biomedical research Areas include: Biochemistry and molecular biology Cell biology Molecular and cellular aspects of biomedicine Neuroscience Pharmacology Immunology Additional Features: Welcomes comments on any article published in CMLS Accepts suggestions for topics to be covered
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