Department of Biochemistry, University of Washington, Seattle, Washington 98195, USA.
Nat Rev Mol Cell Biol. 2010 Aug;11(8):567-78. doi: 10.1038/nrm2944.
One of the many debated topics in ageing research is whether progeroid syndromes are really accelerated forms of human ageing. The answer requires a better understanding of the normal ageing process and the molecular pathology underlying these rare diseases. Exciting recent findings regarding a severe human progeria, Hutchinson-Gilford progeria syndrome, have implicated molecular changes that are also linked to normal ageing, such as genome instability, telomere attrition, premature senescence and defective stem cell homeostasis in disease development. These observations, coupled with genetic studies of longevity, lead to a hypothesis whereby progeria syndromes accelerate a subset of the pathological changes that together drive the normal ageing process.
在衰老研究的众多争议话题中，其中一个是早衰综合征是否真的是人类衰老的加速形式。要回答这个问题，需要更好地了解正常衰老过程和这些罕见疾病的分子病理学。最近关于一种严重的人类早衰症——哈钦森-吉尔福德早衰症的令人兴奋的发现表明，分子变化也与正常衰老有关，如基因组不稳定性、端粒磨损、过早衰老和干细胞功能障碍在疾病发展中的作用。这些观察结果，加上对长寿的遗传研究，提出了一个假说，即早衰综合征加速了共同驱动正常衰老过程的一部分病理变化。
