推进囊性纤维化的防治工作:干细胞和基因疗法的启示。

IF 2 4区 医学 Q3 MEDICINE, RESEARCH & EXPERIMENTAL Current Medical Science Pub Date : 2024-12-01 Epub Date: 2024-12-16 DOI:10.1007/s11596-024-2936-5
Disha D Shah, Mehul R Chorawala, Aanshi J Pandya, Nirjari Kothari, Bhupendra G Prajapati, Priyajeet S Parekh
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引用次数: 0

摘要

囊性纤维化(CF)是一种遗传性疾病,其特点是 CFTR 基因发生突变,导致氯离子转运功能受损,进而导致各器官(尤其是肺部)粘液变稠。尽管在 CF 的治疗方面取得了重大进展,但目前的治疗方法主要集中在缓解症状上,并没有从根本上解决基因缺陷问题。干细胞和基因疗法为从根本上解决 CF 问题提供了前景广阔的途径。干细胞,包括胚胎细胞、诱导多能细胞、间充质细胞、造血细胞和肺祖细胞,通过分化为特化细胞和调节免疫反应,具有再生潜力。同样,基因疗法的目的是通过向受影响的细胞提供功能基因拷贝来纠正 CFTR 基因突变。目前正在探索各种方法来实现基因校正,如病毒和非病毒载体、CRISPR-Cas9 基因编辑、小干扰 RNA(siRNA)疗法和 mRNA疗法。尽管这些疗法潜力巨大,但仍存在安全问题、伦理考虑、传输系统优化和长期疗效等挑战。本综述全面概述了目前对CF病理生理学的理解、探索干细胞和基因疗法的理由、现有疗法的类型、作用机制以及该领域的挑战和未来方向。通过应对这些挑战,干细胞和基因疗法有望改变CF管理,改善患者的生活质量。
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Advancing the Battle against Cystic Fibrosis: Stem Cell and Gene Therapy Insights.

Cystic fibrosis (CF) is a hereditary disorder characterized by mutations in the CFTR gene, leading to impaired chloride ion transport and subsequent thickening of mucus in various organs, particularly the lungs. Despite significant progress in CF management, current treatments focus mainly on symptom relief and do not address the underlying genetic defects. Stem cell and gene therapies present promising avenues for tackling CF at its root cause. Stem cells, including embryonic, induced pluripotent, mesenchymal, hematopoietic, and lung progenitor cells, offer regenerative potential by differentiating into specialized cells and modulating immune responses. Similarly, gene therapy aims to correct CFTR gene mutations by delivering functional copies of the gene into affected cells. Various approaches, such as viral and nonviral vectors, gene editing with CRISPR-Cas9, small interfering RNA (siRNA) therapy, and mRNA therapy, are being explored to achieve gene correction. Despite their potential, challenges such as safety concerns, ethical considerations, delivery system optimization, and long-term efficacy remain. This review provides a comprehensive overview of the current understanding of CF pathophysiology, the rationale for exploring stem cell and gene therapies, the types of therapies available, their mechanisms of action, and the challenges and future directions in the field. By addressing these challenges, stem cell and gene therapies hold promise for transforming CF management and improving the quality of life of affected individuals.

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来源期刊
Current Medical Science
Current Medical Science Biochemistry, Genetics and Molecular Biology-Genetics
CiteScore
4.70
自引率
0.00%
发文量
126
期刊介绍: Current Medical Science provides a forum for peer-reviewed papers in the medical sciences, to promote academic exchange between Chinese researchers and doctors and their foreign counterparts. The journal covers the subjects of biomedicine such as physiology, biochemistry, molecular biology, pharmacology, pathology and pathophysiology, etc., and clinical research, such as surgery, internal medicine, obstetrics and gynecology, pediatrics and otorhinolaryngology etc. The articles appearing in Current Medical Science are mainly in English, with a very small number of its papers in German, to pay tribute to its German founder. This journal is the only medical periodical in Western languages sponsored by an educational institution located in the central part of China.
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