Defective adhesion of blood platelets to vascular microfibrils in the Bernard-Soulier syndrome

Author:

Fauvel-Lafeve F1,Tabaka V1,Caen JP1,Legrand YJ1

Affiliation:

1. Unite 353 Inserm, Hopital Saint Louis, Paris, France.

Abstract

Abstract Bernard-Soulier syndrome (BSS) platelets, which lack the membrane glycoprotein complex Ib-IX, do not adhere to subendothelium. The adhesion of platelets from two patients with BSS to subendothelial microfibrils (MFs) and type I collagen was compared in an in vitro assay adapted to patients with low platelet count. With both patients, platelet adhesion to MFs was strongly defective, whereas the adhesion to collagen was normal. The involvement of GPIb in the MFs-induced platelet adhesion was confirmed by the inhibitory effect of a MoAb (AN51) to the von Willebrand (vWF) factor binding domain of GPIb. The adhesion of platelets to MFs thus requires GPIb-IX and an axis MFs-vWF- GPIb-IX seems therefore to be prevalent in the reactivity of platelets with subendothelium.

Publisher

American Society of Hematology

Subject

Cell Biology,Hematology,Immunology,Biochemistry

Cited by 6 articles. 订阅此论文施引文献 订阅此论文施引文献,注册后可以免费订阅5篇论文的施引文献,订阅后可以查看论文全部施引文献

1. Inherited giant platelet disorders;European Journal of Haematology;2009-04-24

2. Flow cytometry as a tool in the diagnosis of Bernard-Soulier Syndrome in Brazilian patients;Platelets;2009-01

3. Biochemical hematology of platelets and leukocytes;Advances in Clinical Chemistry;2001

4. Inherited Giant Platelet Disorders;American Journal of Clinical Pathology;2000-02-01

5. Microfibrils from the Arterial Subendothelium;International Review of Cytology;1999

同舟云学术

1.学者识别学者识别

2.学术分析学术分析

3.人才评估人才评估

"同舟云学术"是以全球学者为主线,采集、加工和组织学术论文而形成的新型学术文献查询和分析系统,可以对全球学者进行文献检索和人才价值评估。用户可以通过关注某些学科领域的顶尖人物而持续追踪该领域的学科进展和研究前沿。经过近期的数据扩容,当前同舟云学术共收录了国内外主流学术期刊6万余种,收集的期刊论文及会议论文总量共计约1.5亿篇,并以每天添加12000余篇中外论文的速度递增。我们也可以为用户提供个性化、定制化的学者数据。欢迎来电咨询!咨询电话:010-8811{复制后删除}0370

www.globalauthorid.com

TOP

Copyright © 2019-2024 北京同舟云网络信息技术有限公司
京公网安备11010802033243号  京ICP备18003416号-3