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超越疾病到功能:多聚谷氨酰胺序列在细胞决策中的生理作用。

Moving beyond disease to function: Physiological roles for polyglutamine-rich sequences in cell decisions.

机构信息

Department of Biological Sciences, University of North Carolina at Chapel Hill, Chapel Hill, NC, 27599, USA.

Department of Biological Sciences, University of North Carolina at Chapel Hill, Chapel Hill, NC, 27599, USA; Marine Biological Laboratory, Woods Hole, MA, 02543, USA.

出版信息

Curr Opin Cell Biol. 2021 Apr;69:120-126. doi: 10.1016/j.ceb.2021.01.003. Epub 2021 Feb 18.

Abstract

Glutamine-rich tracts, also known as polyQ domains, have received a great deal of attention for their role in multiple neurodegenerative diseases, including Huntington's disease (HD), spinocerebellar ataxia (SCA), and others [22], [27]. Expansions in the normal polyQ tracts are thus commonly linked to disease, but polyQ domains themselves play multiple important functional roles in cells that are being increasingly appreciated. The biochemical nature of these domains allows them to adopt a number of different structures and form large assemblies that enable environmental responsiveness, localized signaling, and cellular memory. In many cases, these involve the formation of condensates that have varied material states. In this review, we highlight known and emerging functional roles for polyQ tracts in normal cell physiology.

摘要

富含谷氨酰胺的区域,也称为多聚谷氨酰胺区,因其在多种神经退行性疾病中的作用而受到广泛关注,包括亨廷顿病 (HD)、脊髓小脑共济失调 (SCA) 等[22],[27]。正常多聚谷氨酰胺区的扩展通常与疾病有关,但多聚谷氨酰胺区本身在细胞中具有多种重要的功能作用,这一点正越来越受到重视。这些结构域的生化性质使它们能够采用多种不同的结构,并形成允许环境响应、局部信号传递和细胞记忆的大型组装体。在许多情况下,这些涉及到具有不同物质状态的凝聚体的形成。在这篇综述中,我们强调了多聚谷氨酰胺区在正常细胞生理学中的已知和新兴功能作用。

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