Miller Jonathan L
Medical Center Laboratories, The University of Chicago, 5841 S. Maryland Avenue, Chicago, IL 60637, USA.
Semin Thromb Hemost. 2009 Mar;35(2):224-32. doi: 10.1055/s-0029-1220330. Epub 2009 Apr 30.
Platelet glycoproteins subserve a wide variety of critical functions in blood platelets. Congenital deficiencies or functional abnormalities in platelet glycoproteins may produce serious bleeding disorders such as Glanzmann thrombasthenia or Bernard-Soulier syndrome. Other hematologic disorders, such as Fanconi anemia and various myelodysplastic syndromes, may also be associated with abnormalities in platelet glycoproteins. Additionally, several acquired disorders involving the major platelet glycoproteins are increasingly being recognized. The large number of techniques, now available to characterize platelet glycoprotein disorders, reflect the many advances in biochemistry, molecular analysis, flow cytometry, and, most recently, proteomics. The application of platelet glycoprotein analysis to a wide range of clinical disorders is reviewed in this article.
血小板糖蛋白在血小板中发挥着多种关键功能。血小板糖蛋白的先天性缺陷或功能异常可能导致严重的出血性疾病,如Glanzmann血小板无力症或Bernard-Soulier综合征。其他血液系统疾病,如范可尼贫血和各种骨髓增生异常综合征,也可能与血小板糖蛋白异常有关。此外,越来越多涉及主要血小板糖蛋白的后天性疾病被发现。目前用于表征血小板糖蛋白疾病的大量技术反映了生物化学、分子分析、流式细胞术以及最近的蛋白质组学等方面的诸多进展。本文综述了血小板糖蛋白分析在广泛临床疾病中的应用。