Mutations in Hsp90 Cochaperones Result in a Wide Variety of Human Disorders.
Mutations in Hsp90 Cochaperones Result in a Wide Variety of Human Disorders.
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DOI：
10.3389/fmolb.2021.787260
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发表时间：
2021
影响因子：
5
通讯作者：
Johnson JL
中科院分区：
文献类型：
--
作者：
Johnson JL
热休克蛋白90分子伴侣，沿着一组约50辅伴侣，介导折叠和激活的数百种细胞蛋白在ATP依赖性循环。Cochaperones的不同之处在于它们如何与Hsp 90相互作用以及它们调节Hsp 90的ATP酶活性的能力。辅伴侣蛋白通常竞争Hsp 90上的相同结合位点，并且在神经变性、癌症或衰老期间发生的辅伴侣蛋白表达水平的变化可能导致改变的Hsp 90-辅伴侣蛋白复合物和客户活动。这篇综述总结了个别辅伴侣蛋白功能缺失突变的信息，并讨论了辅伴侣蛋白改变与广泛疾病的整体关联。辅伴侣突变导致睫状体或肌肉缺陷、神经发育或变性障碍以及其他障碍。在许多情况下，疾病与既定的伴侣-客户互动中的缺陷有关。更好地了解缺陷cochaperones的功能后果将提供新的见解，他们的功能，并可能导致专门的方法来调节热休克蛋白90的功能和治疗这些人类疾病。
The Hsp90 molecular chaperone, along with a set of approximately 50 cochaperones, mediates the folding and activation of hundreds of cellular proteins in an ATP-dependent cycle. Cochaperones differ in how they interact with Hsp90 and their ability to modulate ATPase activity of Hsp90. Cochaperones often compete for the same binding site on Hsp90, and changes in levels of cochaperone expression that occur during neurodegeneration, cancer, or aging may result in altered Hsp90-cochaperone complexes and client activity. This review summarizes information about loss-of-function mutations of individual cochaperones and discusses the overall association of cochaperone alterations with a broad range of diseases. Cochaperone mutations result in ciliary or muscle defects, neurological development or degeneration disorders, and other disorders. In many cases, diseases were linked to defects in established cochaperone-client interactions. A better understanding of the functional consequences of defective cochaperones will provide new insights into their functions and may lead to specialized approaches to modulate Hsp90 functions and treat some of these human disorders.
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