基于干细胞/祖细胞的治疗杜氏肌营养不良症。

IF 4.6 2区 生物学 Q2 CELL BIOLOGY
Frontiers in Cell and Developmental Biology Pub Date : 2025-09-03 eCollection Date: 2025-01-01 DOI:10.3389/fcell.2025.1640275
Tsukasa Tominari, Chaitra Sathyaprakash, Yoshitsugu Aoki
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引用次数: 0

摘要

杜氏肌营养不良症是一种遗传性疾病,其中肌基质相关蛋白(肌营养不良蛋白)的丧失导致进行性肌肉萎缩,并最终因与心脏缺陷相关的并发症而丧失生命。目前,许多恢复肌营养不良蛋白的分子疗法已进入临床试验阶段。然而,这些策略在促进组织再生和减少纤维化方面的治疗效益仍然有限。干细胞/祖细胞为基础的治疗DMD患者是一个很有前途的策略,以促进肌肉再生,尽管移植的条件和许多细胞类型的预处理仍在优化。几种具有不同性质和来源的细胞类型,如肌源性干细胞/祖细胞、间充质干细胞(MSCs)和诱导多能干细胞(iPSCs),已被研究用于治疗DMD。来源于健康供体的肌源性干细胞/祖细胞有望恢复DMD肌肉中肌纤维的数量以及肌营养不良蛋白的表达。来自各种组织(包括脐带)的间质干细胞具有免疫抑制特性,并有望与其他基因疗法联合改善DMD表型。在这篇综述中,我们将总结成功的DMD肌肉组织再生必须克服的挑战,并回顾基于干细胞/祖细胞的DMD治疗的最新发现。我们将重点关注细胞的预处理替代疗法和治疗疾病的生态位,以改善肌纤维的整合。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Stem/progenitor cell-based therapy for Duchenne muscular dystrophy.

Duchenne muscular dystrophy is a genetic disease where loss of sarcolemma-associated protein, dystrophin, leads to progressive muscle wasting, and eventual loss of life from complications linked to cardiac deficits. Currently, numerous molecular therapies to restore dystrophin have entered clinical trials. However, the therapeutic benefits of these strategies in promoting tissue regeneration and reducing fibrosis remain limited. Stem/progenitor cell-based therapy in DMD patients is a promising strategy to promote muscle regeneration, though the conditions of transplantation and pre-treatments of numerous cell types are still being optimized. Several cell types with different properties and origins, such as myogenic stem/progenitor cells, mesenchymal stem cells (MSCs), and induced pluripotent stem cells (iPSCs), have been studied for treating DMD. Myogenic stem/progenitor cells derived from healthy donors are expected to restore the number of myofibers as well as dystrophin expression in DMD muscles. MSCs derived from various tissues, including umbilical cord, have immunosuppressive properties and are expected to ameliorate DMD phenotypes in combination with other gene therapies. In this review, we will summarize the challenges that must be overcome to allow for successful DMD muscle tissue regeneration and review the latest findings in stem/progenitor cell-based DMD therapy. We will focus on the pre-conditioning of cells for replacement therapies and treatment of the disease niche to improve muscle fiber integration.

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来源期刊
Frontiers in Cell and Developmental Biology
Frontiers in Cell and Developmental Biology Biochemistry, Genetics and Molecular Biology-Cell Biology
CiteScore
9.70
自引率
3.60%
发文量
2531
审稿时长
12 weeks
期刊介绍: Frontiers in Cell and Developmental Biology is a broad-scope, interdisciplinary open-access journal, focusing on the fundamental processes of life, led by Prof Amanda Fisher and supported by a geographically diverse, high-quality editorial board. The journal welcomes submissions on a wide spectrum of cell and developmental biology, covering intracellular and extracellular dynamics, with sections focusing on signaling, adhesion, migration, cell death and survival and membrane trafficking. Additionally, the journal offers sections dedicated to the cutting edge of fundamental and translational research in molecular medicine and stem cell biology. With a collaborative, rigorous and transparent peer-review, the journal produces the highest scientific quality in both fundamental and applied research, and advanced article level metrics measure the real-time impact and influence of each publication.
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