《血管性血友病因子多聚体组装和WP小体储存的结构机制》解读

2022-08-20 上海交通大学医学院附属瑞金医院检验科 血栓与止血学

本文于2022年6月发表于《Blood》杂志,发表后引起业内广泛关注,在Blood Podcast (Episode 22Season 3, https://ashpublications.org/b

中文标题:

《血管性血友病因子多聚体组装和WP小体储存的结构机制》解读

发布日期:

2022-08-20

简要介绍:

本文于2022年6月发表于《Blood》杂志,发表后引起业内广泛关注,在Blood Podcast (Episode 22Season 3, https://ashpublications.org/blood/pages/blood_podcast)和《JTH》杂志What The Neighbors Say栏目(J Thromb Haemost,2022,20:1036)重点介绍。本文文章题录为:Zeng JW,Shu ZM,Liang Q,Zhang J,Wu WM,Wang XF,Zhou AW. Structural basis of Von Willebrand Factor multimerization and tubular storage,Blood,2022 Jun 2;139(22):3314-3324.该研究首次通过单颗粒冷冻电镜技术获得了高分辨率的由VWF N端结构域(D1D2D’D3结构域)形成的管状多聚体结构,揭示了VWF多聚体组装和Weibel-Palade小体储存的结构机制。该理论不仅解开了VWF多聚体组装和储存机制的谜团,也为2A型血管性血友病(VWD)患者基因治疗新策略奠定了理论基础。经通信作者许可,再次通过佳文解读的方式来阐述这一发现。

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