Hierarchical deterioration of body systems in Werner's syndrome: Implications for normal ageing
Hierarchical deterioration of body systems in Werner's syndrome: Implications for normal ageing
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DOI：
10.1016/s0047-6374(97)00111-5
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发表时间：
1997-12-01
影响因子：
5.3
通讯作者：
Goto, M
中科院分区：
文献类型：
--
作者：
Goto, M
人类的正常衰老是一个复杂的生物学现象。由RecQ型DNA解旋酶突变引起的“Werner's综合征（WS Ⅱ）”被认为是一种顶级的“节段性”早衰样综合征。WS患者在其生命的早期阶段在四个主要自组装身体系统（神经、免疫、结缔组织和内分泌代谢系统）中显示出与正常衰老相似的各种临床和生物学表现，随后在平均46岁时死亡。在WS中观察到的身体系统的临床和生物学恶化的连续出现表明，该疾病不仅仅是节段性早老综合征，对其进行分析可能会对“我们为什么以及如何衰老”的问题提供新的见解。'（C）1997 Elsevier Science爱尔兰有限公司
Normal human ageing is a complicated biological phenomenon. 'Werner's syndrome (WS)', caused by mutations of RecQ type DNA helicase, has been recognized as a top ranking 'segmental' progeroid syndrome. Patients with WS show a wide variety of clinical and biological manifestations in the four major self-assembly body systems (nervous, immune, connective tissue and endocrine-metabolic systems) similar to normal ageing at an early stage of their life, followed by death at an average age of 46. The sequential appearance of clinical and biological deterioration of the body systems observed in WS suggested that the disorder is more than a segmental progeroid syndrome, analysis of which may shed new light on the question 'Why and how we age?' (C) 1997 Elsevier Science Ireland Ltd.