New insights of glycoprotein Ib-IX-V complex organization and glycoprotein Ibα in platelet biogenesis.

IF 3.1 3区医学 Q2 HEMATOLOGY Current Opinion in Hematology Pub Date : 2024-11-01 Epub Date: 2024-07-16 DOI:10.1097/MOH.0000000000000832

Lulu Huang, Bojing Shao

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Abstract

Purpose of review: Glycoprotein (GP) Ib-IX-V, a platelet surface receptor that plays a critical role in platelet adhesion and platelet-mediated immune responses, consists of GPIbα, GPIbβ, GPIX, and GPV in a stoichiometry of 2 : 4 : 2 : 1. Forming a complex is essential for GPIb-IX-V to function. GPIb-IX-V also plays an important role in platelet biogenesis by regulating the number and size of platelets. Yet how GPIb-IX-V regulates platelet biogenesis remains elusive. This review will summarize recent findings in the complex organization of GPIb-IX-V and its role in platelet biogenesis.

Recent findings: Proteomics studies suggest that GPIbα, GPIbβ, GPIX, and GPV form the complex in a ratio of 1 : 2 : 1 : 1, which is supported by analysis of molecular weight of GPIb-IX-V and GPIb-IX and the structure of entire GPIb-IX-V. To activate platelets, GPIbα requires binding of CLEC-2 to trigger signals. Furthermore, disrupting the GPIbα anchorage to filamin A causes defects in platelet budding away from proplatelets leading to giant platelets and a low platelet count.

Summary: New studies challenge the traditional model for the organization of GPIb-IX-V as a complex and indicate the role of GPIb-IX-V in platelet production. Those studies provide insights for GPIb-IX-V in the regulation of platelet activation and platelet biogenesis.

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糖蛋白 Ib-IX-V 复合物组织和糖蛋白 Ibα 在血小板生物生成过程中的新见解。

综述目的：糖蛋白（GP）Ib-IX-V 是一种血小板表面受体，在血小板粘附和血小板介导的免疫反应中发挥着关键作用，它由 GPIbα、GPIbβ、GPIX 和 GPV 组成，其化学计量为 2 ：4 : 2 :1.形成复合物对 GPIb-IX-V 发挥作用至关重要。GPIb-IX-V 还通过调节血小板的数量和大小，在血小板的生物生成过程中发挥重要作用。然而，GPIb-IX-V 如何调节血小板的生物生成仍是一个谜。本综述将总结有关 GPIb-IX-V 复杂组织及其在血小板生物生成中作用的最新发现：蛋白质组学研究表明，GPIbα、GPIbβ、GPIX 和 GPV 以 1 : 2 的比例形成复合物：1 :GPIb-IX-V 和 GPIb-IX 的分子量分析以及整个 GPIb-IX-V 的结构都证明了这一点。要激活血小板，GPIbα 需要与 CLEC-2 结合才能触发信号。此外，破坏 GPIbα 与丝胺 A 的锚定会导致血小板从原血小板上出芽的缺陷，从而导致巨型血小板和低血小板计数。摘要：新的研究挑战了 GPIb-IX-V 作为复合物的传统组织模型，并指出了 GPIb-IX-V 在血小板生成中的作用。这些研究为 GPIb-IX-V 在血小板活化和血小板生物生成调控中的作用提供了启示。

本文章由计算机程序翻译，如有差异，请以英文原文为准。

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来源期刊

Current Opinion in Hematology 医学-血液学

CiteScore

6.60

自引率

3.10%

发文量

审稿时长

6-12 weeks

期刊介绍： Current Opinion in Hematology is an easy-to-digest bimonthly journal covering the most interesting and important advances in the field of hematology. Its hand-picked selection of editors ensure the highest quality selection of unbiased review articles on themes from nine key subject areas, including myeloid biology, Vascular biology, hematopoiesis and erythroid system and its diseases.