Eaton L A, Read M S, Brinkhous K M
Department of Pathology, University of North Carolina, Chapel Hill 27599.
Arch Pathol Lab Med. 1991 May;115(5):488-93.
Glycoprotein Ib (GP-Ib) is a major platelet receptor protein concerned with von Willebrand-factor binding, platelet agglutination, and platelet adhesion, and it is required for normal hemostasis. By the use of botrocetin (venom coagglutinin), both quantitative and semiquantitative assays for GP-Ib activity were developed. The latter assay uses limiting dilutions of botrocetin as a measure of GP-Ib activity. Platelets, stored up to 23 days under blood bank conditions, were assayed by the limiting dilution test. Values of GP-Ib were progressively diminished after 9 to 10 days of storage, reaching levels of less than 10% at 23 days. Platelets from a subject with Bernard-Soulier syndrome showed less than 10% GP-Ib activity. These assays appear to be a specific measure of functional GP-Ib activity, and, when combined with GP-Ib antigen measurement by other methods, they provide a means for further characterizing GP-Ib abnormalities.
糖蛋白 Ib（GP-Ib）是一种主要的血小板受体蛋白，与血管性血友病因子结合、血小板凝集和血小板黏附有关，是正常止血所必需的。通过使用蛇毒凝血素（毒液凝集素），开发了GP-Ib活性的定量和半定量检测方法。后一种检测方法使用有限稀释的蛇毒凝血素作为GP-Ib活性的指标。在血库条件下储存长达23天的血小板，通过有限稀释试验进行检测。储存9至10天后，GP-Ib值逐渐降低，在23天时降至不到10%的水平。患有伯纳德-索利尔综合征的受试者的血小板显示GP-Ib活性低于10%。这些检测似乎是功能性GP-Ib活性的一种特异性指标，并且当与通过其他方法进行的GP-Ib抗原测量相结合时，它们为进一步表征GP-Ib异常提供了一种手段。
