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spectrins 与人类疾病。

Spectrins and human diseases.

机构信息

The First School of Clinical Medicine, Lanzhou University, Gansu, China.

Central Laboratory, The First Hospital of Lanzhou University, Gansu, China.

出版信息

Transl Res. 2022 May;243:78-88. doi: 10.1016/j.trsl.2021.12.009. Epub 2021 Dec 31.

Abstract

Spectrin, as one of the major components of a plasma membrane-associated cytoskeleton, is a cytoskeletal protein composed of the modular structure of α and β subunits. The spectrin-based skeleton is essential for preserving the integrity and mechanical characteristics of the cell membrane. Moreover, spectrin regulates a variety of cell processes including cell apoptosis, cell adhesion, cell spreading, and cell cycle. Dysfunction of spectrins is implicated in various human diseases including hemolytic anemia, neurodegenerative diseases, ataxia, heart diseases, and cancers. Here, we briefly discuss spectrins function as well as the clinical manifestations and currently known molecular mechanisms of human diseases related to spectrins, highlighting that strategies for targeting regulation of spectrins function may provide new avenues for therapeutic intervention for these diseases.

摘要

血影蛋白是一种与质膜相关的细胞骨架的主要成分,属于细胞骨架蛋白,由α和β亚基的模块结构组成。基于血影蛋白的骨架对于维持细胞膜的完整性和机械特性至关重要。此外,血影蛋白还调节多种细胞过程,包括细胞凋亡、细胞黏附、细胞铺展和细胞周期。血影蛋白功能障碍与多种人类疾病有关,包括溶血性贫血、神经退行性疾病、共济失调、心脏病和癌症。在这里,我们简要讨论了血影蛋白的功能以及与血影蛋白相关的人类疾病的临床表现和目前已知的分子机制,强调了靶向调节血影蛋白功能的策略可能为这些疾病的治疗干预提供新的途径。

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