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血管性血友病因子抗原II。一种在严重血管性血友病中缺乏的新型血浆和血小板抗原。

von Willebrand's disease antigen II. A new plasma and platelet antigen deficient in severe von Willebrand's disease.

作者信息

Montgomery R R, Zimmerman T S

出版信息

J Clin Invest. 1978 Jun;61(6):1498-507. doi: 10.1172/JCI109070.

Abstract

Factor VIII-related antigen (VIIIag) is deficient in plasma and platelets of patients with severe von Willebrand's disease. This study reports a second von Willebrand's disease antigen (vWagII), distinct from VIIIag, that is also deficient in the platelets and plasma of patients with severe von Willebrand's disease. VIIIag and vWagII are separable by molecular exclusion chromatography, sucrose density gradient ultracentrifugation, and crossed immunoelectrophoresis. They show reactions of immunologic nonidentity with each other, and thus, do not share a precursor-product relationship. vWagII is released from normal platelets during blood clotting, accounting for a fourfold higher concentration of vWagII in serum over plasma.

摘要

重度血管性血友病患者的血浆和血小板中缺乏Ⅷ因子相关抗原(VIIIag)。本研究报告了一种不同于VIIIag的第二种血管性血友病抗原(vWagII),重度血管性血友病患者的血小板和血浆中也缺乏该抗原。VIIIag和vWagII可通过分子排阻色谱法、蔗糖密度梯度超速离心法和交叉免疫电泳法分离。它们彼此之间表现出免疫非同一性反应,因此不存在前体-产物关系。vWagII在血液凝固过程中从正常血小板中释放出来,导致血清中vWagII的浓度比血浆中高四倍。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/dbcb/372676/654cb56943f7/jcinvest00666-0085-a.jpg

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