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推进对抗囊性纤维化的战斗:干细胞与基因治疗见解

Advancing the Battle against Cystic Fibrosis: Stem Cell and Gene Therapy Insights.

作者信息

Shah Disha D, Chorawala Mehul R, Pandya Aanshi J, Kothari Nirjari, Prajapati Bhupendra G, Parekh Priyajeet S

机构信息

Department of Pharmacology and Pharmacy Practice, L. M. College of Pharmacy, Opp. Gujarat University, Navrangpura, Ahmedabad, 380009, India.

Department of Pharmaceutics and Pharmaceutical Technology, Shree S. K. Patel College of Pharmaceutical Education & Research, Ganpat University, Mehsana, 384012, India.

出版信息

Curr Med Sci. 2024 Dec;44(6):1155-1174. doi: 10.1007/s11596-024-2936-5. Epub 2024 Dec 16.

DOI:10.1007/s11596-024-2936-5
PMID:39676146
Abstract

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.

摘要

囊性纤维化(CF)是一种遗传性疾病,其特征在于CFTR基因突变,导致氯离子转运受损,进而使各个器官(尤其是肺部)的黏液增厚。尽管在CF治疗方面取得了重大进展,但目前的治疗主要集中在缓解症状,并未解决潜在的基因缺陷。干细胞和基因疗法为从根本上治疗CF提供了有前景的途径。干细胞包括胚胎干细胞、诱导多能干细胞、间充质干细胞、造血干细胞和肺祖细胞,通过分化为特化细胞和调节免疫反应具有再生潜力。同样,基因疗法旨在通过将该基因的功能拷贝递送至受影响的细胞来纠正CFTR基因突变。正在探索各种方法,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,以实现基因校正。尽管它们具有潜力,但仍存在安全问题、伦理考量、递送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基本原理、可用疗法的类型、它们的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的治疗方式并改善受影响个体的生活质量。

相似文献

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Advancing the Battle against Cystic Fibrosis: Stem Cell and Gene Therapy Insights.推进对抗囊性纤维化的战斗:干细胞与基因治疗见解
Curr Med Sci. 2024 Dec;44(6):1155-1174. doi: 10.1007/s11596-024-2936-5. Epub 2024 Dec 16.
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Targeting the Root Cause of Cystic Fibrosis.针对囊性纤维化的根本原因。
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