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协助折叠致病的质膜蛋白。

Assistance for Folding of Disease-Causing Plasma Membrane Proteins.

机构信息

Facultad de Ciencias Quimicas, Universidad Juarez del Estado de Durango, Durango C.P 34000, Mexico.

出版信息

Biomolecules. 2020 May 7;10(5):728. doi: 10.3390/biom10050728.

Abstract

An extensive catalog of plasma membrane (PM) protein mutations related to phenotypic diseases is associated with incorrect protein folding and/or localization. These impairments, in addition to dysfunction, frequently promote protein aggregation, which can be detrimental to cells. Here, we review PM protein processing, from protein synthesis in the endoplasmic reticulum to delivery to the PM, stressing the main repercussions of processing failures and their physiological consequences in pathologies, and we summarize the recent proposed therapeutic strategies to rescue misassembled proteins through different types of chaperones and/or small molecule drugs that safeguard protein quality control and regulate proteostasis.

摘要

大量与表型疾病相关的质膜(PM)蛋白突变与错误的蛋白质折叠和/或定位有关。这些损伤除了功能障碍外,还经常促进蛋白质聚集,这对细胞是有害的。在这里,我们回顾了 PM 蛋白的加工过程,从内质网中的蛋白质合成到递送到 PM,强调了加工失败的主要影响及其在疾病中的生理后果,并总结了最近提出的通过不同类型的伴侣蛋白和/或小分子药物来拯救错误组装的蛋白质的治疗策略,这些药物可以保护蛋白质质量控制并调节蛋白质平衡。

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