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Getting out of shape

机译：变形

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摘要

Two greatly debilitating illnesses ―Alzheimer's disease, which is largely associated with ageing, and variant Creutzfeldt-Jakob disease (vCJD), which is linked with the recent bovine spongiform encephalopathy (BSE) epidemic in Britain ― have had a huge impact on public consciousness. Despite their very different origins, these diseases are closely related to each other and also to the 20 or so other 'amyloidoses' ― so called because they involve the aberrant deposition of proteins in the form of amyloid fibrils or plaques. Other diseases in this group, which are less publicized but no less devastating to those affected, are Parkinson's disease, type II (late-onset) diabetes and rare conditions such as familial insomnia. Amyloidoses thus include some of the most feared and costly diseases in the Western world. Alzheimer's may very soon be the most prevalent and socially disruptive illness in the ageing populations of all developed countries. It is remarkable, therefore, that most of these diseases were virtually unknown until relatively recently. Indeed, the first detailed description of an amyloid pathology was made less than 100 years ago, when Alzheimer identified the form of dementia that bears his name. As with many amyloidoses, Alzheimer's disease is usually sporadic, although there are less common hereditary forms that can afflict relatively young people ― Alzheimer's most famous patient was in her early 50s.
机译：两种严重使人衰弱的疾病-与衰老有关的阿尔茨海默氏病和与最近在英国流行的牛海绵状脑病（BSE）相关的变异性克雅兹病（vCJD）-对公众的意识产生了巨大影响。尽管它们的起源非常不同，但它们彼此之间以及与20多种其他“淀粉样糖”密切相关-之所以被称为“淀粉样糖”，是因为它们涉及淀粉样蛋白原纤维或斑块形式的蛋白质异常沉积。该组中的其他疾病对帕金森氏病，II型（迟发性）糖尿病和罕见疾病（如家族性失眠）的报道较少，但对受影响的人群却没有造成破坏性的破坏。因此，淀粉样糖包括西方世界中一些最令人恐惧和代价最高的疾病。在所有发达国家的老龄化人口中，阿尔茨海默氏病可能很快就会成为最普遍和社会破坏性疾病。因此，值得注意的是，直到最近，这些疾病中的大多数实际上还是未知的。的确，淀粉样蛋白病理学的第一个详细描述是在不到100年前进行的，当时阿尔茨海默氏症发现了以他的名字命名的痴呆症形式。与许多淀粉样蛋白一样，阿尔茨海默氏病通常是散发性疾病，尽管不常见的遗传形式会折磨相对年轻的人-阿尔茨海默氏症最著名的患者是50多岁的早期患者。

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  • 来源
    《Nature》 |2002年第6899期|p.729-730|共2页
  • 作者

    Christopher M. Dobson;

  • 作者单位

    Department of Chemistry, University of Cambridge, Lensfield Road, Cambridge CB2 1EW, UK;

  • 收录信息 美国《科学引文索引》(SCI);美国《工程索引》(EI);美国《生物学医学文摘》(MEDLINE);美国《化学文摘》(CA);
  • 原文格式 PDF
  • 正文语种 eng
  • 中图分类 自然科学总论;
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