Chaperonopathies by defect, excess, or mistake
Chaperonopathies by defect, excess, or mistake
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DOI：
10.1196/annals.1391.009
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发表时间：
2007-01-01
期刊：
影响因子：
--
通讯作者：
De Macario, Everly Conway
中科院分区：
文献类型：
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作者：
Macario, Alberto J. L.;De Macario, Everly Conway
在过去的几十年里，人们对应激反应、应激蛋白、热休克基因和蛋白、分子伴侣基因和蛋白以及许多密切相关的分子和细胞过程进行了研究。大量的信息已经积累，分散在印刷和电子文献和数据库中。这些信息中的大部分构成了陪伴科学的主题。最近，分子伴侣病理学的概念，生病的分子伴侣，已经发展，因为各种病理条件已被确定，其中有缺陷的分子伴侣发挥病因学的作用。这些情况是伴侣病。本文简要讨论了伴侣病的最新研究结果。伴侣蛋白病发生在所有年龄段;作为一项规则，遗传性病例具有早期临床发作，而获得性伴侣蛋白病在老年人中变得明显和/或与其他疾病相关。在不久的将来最有可能扩展的伴侣学的其他领域是细胞外伴侣、伴侣网络、伴侣的治疗用途（即，分子伴侣疗法（chaperonotherapy）以管理分子伴侣病和改善细胞在面对压力时的表现，评价分子伴侣作为诊断标志物和作为预后指标，以及开发抗分子伴侣剂以在分子伴侣导致疾病而不是相反时抑制分子伴侣基因表达或抑制分子伴侣功能。
The stress response, stress proteins, heat-shock genes and proteins, molecular chaperone genes and proteins, and a number of closely related molecules and cellular processes have been studied over the last few decades. A huge amount of information has accumulated that is scattered in printed and electronic literature and databases. Most of this information constitutes the subject matter of the science of chaperonology. More recently, the concept of chaperone pathology, sick chaperones, has evolved since various pathological conditions have been identified in which defective chaperones play an etiologic role. These conditions are the chaperonopathies. Recent findings on chaperonopathies are briefly discussed in this article. Chaperonopathies occur at all ages; as a rule the genetic cases have an early clinical onset while the acquired chaperonopathies become manifest in the elderly and/or in association with other diseases. Other fields of chaperonology, which will most likely be expanded in the near future, are the study of extracellular chaperones, chaperone networks, the therapeutic use of chaperones (i.e., chaperonotherapy) to manage chaperonopathies and to improve cell performance in the face of stress, the evaluation of chaperones as diagnostic markers and as prognostic indicators, and the development of antichaperone agents to suppress chaperone-gene expression or inhibit chaperone function when chaperones contribute to disease rather than the opposite.