Wellcome Trust Centre for Cell Biology, School of Biological Sciences, University of Edinburgh, Edinburgh EH9 3BF, UK.
Open Biol. 2024 Jun;14(6):240041. doi: 10.1098/rsob.240041. Epub 2024 Jun 5.
Platelets are blood cells derived from megakaryocytes that play a central role in regulating haemostasis and vascular integrity. The microtubule cytoskeleton of megakaryocytes undergoes a critical dynamic reorganization during cycles of endomitosis and platelet biogenesis. Quiescent platelets have a discoid shape maintained by a marginal band composed of microtubule bundles, which undergoes remarkable remodelling during platelet activation, driving shape change and platelet function. Disrupting or enhancing this process can cause platelet dysfunction such as bleeding disorders or thrombosis. However, little is known about the molecular mechanisms underlying the reorganization of the cytoskeleton in the platelet lineage. Recent studies indicate that the emergence of a unique platelet tubulin code and specific pathogenic tubulin mutations cause platelet defects and bleeding disorders. Frequently, these mutations exhibit dominant negative effects, offering valuable insights into both platelet disease mechanisms and the functioning of tubulins. This review will highlight our current understanding of the role of the microtubule cytoskeleton in the life and death of platelets, along with its relevance to platelet disorders.
血小板是由巨核细胞衍生而来的血细胞,在调节止血和血管完整性方面发挥着核心作用。巨核细胞的微管细胞骨架在有丝分裂和血小板生成的循环中经历关键的动态重排。静止的血小板呈圆盘状,由微管束组成的边缘带维持,在血小板激活过程中会发生显著的重塑,驱动形状变化和血小板功能。破坏或增强这个过程会导致血小板功能障碍,如出血性疾病或血栓形成。然而,对于血小板谱系中细胞骨架的重排的分子机制知之甚少。最近的研究表明,独特的血小板微管蛋白编码和特定的致病变异微管蛋白的出现导致血小板缺陷和出血性疾病。这些突变通常表现出显性负效应,为血小板疾病机制和微管蛋白的功能提供了有价值的见解。这篇综述将重点介绍我们目前对微管细胞骨架在血小板生与死中的作用的理解,以及其与血小板疾病的相关性。