端粒酶在发育和癌症中的生物发生和调控。

IF 6.9 2区 生物学 Q1 CELL BIOLOGY
Lu Chen, Luis Francisco Zirnberger Batista
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引用次数: 0

摘要

端粒酶是一种大型核糖核蛋白复合物,负责将端粒DNA重复序列添加到染色体末端。端粒酶由核心和辅助成分组成,它们协调工作以确保端粒在发育过程中和特定细胞类型中保持长度。端粒酶活性在大多数肿瘤细胞中受到严格调控,并显著增加。另一方面,无论是辅助因子还是复杂的核心成分的功能丧失突变都会影响端粒的维持,并导致大范围的严重表型,通常被描述为端粒生物学紊乱。高效端粒酶功能的核心要素是全酶的适当生物发生和组装。在这里,我们讨论了我们目前对这些过程的理解以及它们如何调节端粒酶的效率。我们考虑在全酶组装的不同阶段,不同端粒酶组分的特定亚细胞定位如何影响这些过程。我们描述了过去十年来在这一领域取得的巨大进展,以及最近发现的端粒酶生物发生方面如何在临床上被利用,以积极地造福端粒生物学疾病的患者。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Biogenesis and Regulation of Telomerase during Development and Cancer.

Telomerase is a large ribonucleoprotein complex responsible for the addition of telomeric DNA repeats to chromosomal ends. Telomerase is composed of core and accessory components that work in coordination to ensure telomere length is maintained during development and in specific cell types. Telomerase activity is tightly regulated and is strongly increased in most tumor cells. On the other hand, loss-of-function mutations either in accessory factors or in core components of the complex impact telomere maintenance and cause a large spectrum of severe phenotypes, typically described as telomere biology disorders. A central element for efficient telomerase function is the proper biogenesis and assembly of the holoenzyme. Here, we discuss our current understanding of these processes and how they modulate telomerase efficiency. We consider how these processes are influenced by the specific subcellular localization of different telomerase components during different stages of the assembly of the holoenzyme. We describe the tremendous progress made in this area over the last decade and how recently discovered aspects of telomerase biogenesis can be exploited clinically, to actively benefit patients suffering from telomere biology disorders.

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来源期刊
CiteScore
15.00
自引率
1.40%
发文量
56
审稿时长
3-8 weeks
期刊介绍: Cold Spring Harbor Perspectives in Biology offers a comprehensive platform in the molecular life sciences, featuring reviews that span molecular, cell, and developmental biology, genetics, neuroscience, immunology, cancer biology, and molecular pathology. This online publication provides in-depth insights into various topics, making it a valuable resource for those engaged in diverse aspects of biological research.
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