Australian Institute for Bioengineering and Nanotechnology, The University of Queensland, Queensland 4072, Australia.
Australian Institute for Bioengineering and Nanotechnology, The University of Queensland, Queensland 4072, Australia.
Ageing Res Rev. 2022 Aug;79:101653. doi: 10.1016/j.arr.2022.101653. Epub 2022 May 26.
Ataxia-telangiectasia (A-T) is caused by absence of the catalytic activity of ATM, a protein kinase that plays a central role in the DNA damage response, many branches of cellular metabolism, redox and mitochondrial homeostasis, and cell cycle regulation. A-T is a complex disorder characterized mainly by progressive cerebellar degeneration, immunodeficiency, radiation sensitivity, genome instability, and predisposition to cancer. It is increasingly recognized that the premature aging component of A-T is an important driver of this disease, and A-T is therefore an attractive model to study the aging process. This review outlines the current state of knowledge pertaining to the molecular and cellular signatures of aging in A-T and proposes how these new insights can guide novel therapeutic approaches for A-T.
共济失调毛细血管扩张症（A-T）是由 ATM 缺乏催化活性引起的，ATM 是一种蛋白激酶，在 DNA 损伤反应、细胞代谢的许多分支、氧化还原和线粒体稳态以及细胞周期调控中发挥核心作用。A-T 是一种复杂的疾病，主要表现为进行性小脑变性、免疫缺陷、辐射敏感性、基因组不稳定性和易患癌症。人们越来越认识到，A-T 的过早衰老成分是该疾病的重要驱动因素，因此 A-T 是研究衰老过程的一个有吸引力的模型。这篇综述概述了与 A-T 衰老的分子和细胞特征相关的现有知识，并提出了这些新的见解如何为 A-T 的新治疗方法提供指导。
