关键词: hemoglobinopathy molecular aging oxidative stress sickle cell anemia telomere telomere shortening

来  源:   DOI:10.2147/JBM.S462758   PDF(Pubmed)

Abstract:
Sickle Cell Anemia (SCA) is a hereditary blood disorder characterized by the presence of abnormal hemoglobin, leading to the formation of sickle-shaped red blood cells. While extensive research has unraveled many aspects of the genetic and molecular basis of SCA, the role of telomere dynamics in disease progression remains a relatively unexplored frontier. This review seeks to provide a comprehensive examination of telomere biology within the context of SCA, aiming to elucidate its potential impact on molecular aging and the progression of the disease. The impact of oxidative stress on telomere dynamics in SCA is explored, with a particular focus on how increased reactive oxygen species (ROS) may contribute to accelerated telomere shortening and genomic instability. Furthermore, the potential relationship between telomere dysfunction and cellular senescence in SCA is investigated, shedding light on how telomere dynamics may contribute to the premature aging of cells in this population. The review concludes by summarizing key findings and proposing potential therapeutic strategies targeting telomere dynamics to mitigate disease progression in SCA. It also identifies gaps in current understanding and suggests avenues for future research, emphasizing the importance of further investigating telomere biology to advance our understanding of molecular aging and disease progression in Sickle Cell Anemia. This comprehensive exploration of telomere dynamics in SCA offers insights into potential mechanisms of molecular aging and disease progression, paving the way for targeted therapeutic interventions and improved disease management.
摘要:
镰状细胞性贫血(SCA)是一种遗传性血液疾病,其特征是存在异常血红蛋白,导致镰刀形红细胞的形成。虽然广泛的研究揭示了SCA的遗传和分子基础的许多方面,端粒动力学在疾病进展中的作用仍然是一个相对未被探索的前沿.这篇综述旨在在SCA的背景下提供端粒生物学的全面检查,旨在阐明其对分子衰老和疾病进展的潜在影响。探讨了氧化应激对SCA端粒动力学的影响,特别关注增加的活性氧(ROS)如何导致加速的端粒缩短和基因组不稳定性。此外,研究了SCA中端粒功能障碍与细胞衰老之间的潜在关系,阐明端粒动力学如何导致该群体中细胞的过早衰老。该综述总结了主要发现,并提出了针对端粒动力学的潜在治疗策略,以减轻SCA中的疾病进展。它还确定了当前理解中的差距,并为未来的研究提供了途径。强调进一步研究端粒生物学的重要性,以促进我们对镰状细胞性贫血的分子老化和疾病进展的理解。这种对SCA中端粒动力学的全面探索提供了对分子衰老和疾病进展的潜在机制的见解。为有针对性的治疗干预和改善疾病管理铺平道路。
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