Macario Alberto J L, Conway de Macario Everly
University of Maryland Biotechnology Institute, Center of Marine Biotechnology, 701 E. Pratt Street, Baltimore, MD 21202, USA.
Ann N Y Acad Sci. 2007 Oct;1113:178-91. doi: 10.1196/annals.1391.009. Epub 2007 May 4.
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.
在过去几十年中，人们对应激反应、应激蛋白、热休克基因与蛋白、分子伴侣基因与蛋白以及一些密切相关的分子和细胞过程进行了研究。现已积累了大量信息，这些信息分散在印刷文献、电子文献及数据库中。其中大部分信息构成了伴侣蛋白学的研究内容。最近，随着各种病理状况的发现，即缺陷伴侣蛋白在其中发挥病因学作用，伴侣蛋白病理学（患病的伴侣蛋白）的概念逐渐形成。这些病症就是伴侣蛋白病。本文简要讨论了关于伴侣蛋白病的最新研究发现。伴侣蛋白病可发生于任何年龄段；通常，遗传性病例临床发病较早，而获得性伴侣蛋白病则在老年人中出现和/或与其他疾病相关。伴侣蛋白学的其他领域，很可能在不久的将来得到拓展，包括细胞外伴侣蛋白的研究、伴侣蛋白网络、利用伴侣蛋白进行治疗（即伴侣蛋白疗法）以治疗伴侣蛋白病并在应激状态下改善细胞功能、评估伴侣蛋白作为诊断标志物和预后指标，以及开发抗伴侣蛋白药物，在伴侣蛋白导致疾病而非起相反作用时抑制伴侣蛋白基因表达或抑制伴侣蛋白功能。
