Degos L, Tobelem G, Lethielleux P, Levy-Toledano S, Caen J, Colombani J
Blood. 1977 Nov;50(5):899-903.
An IgG antibody isolated from the serum of a patient with the Bernard-Soulier syndrome induced platelet agglutination in the platelet-rich plasma of 50 normal subjects regardless of their ABO, KOa, KOb, HLA, or PlA1 types. This antibody was nonreactive with platelets from three other Bernard-Saulier syndrome patients. Indirect immunoprecipitation tests using this serum (or purified IgG) and soluble membrane antigens labeled with 125I that had been extracted from normal platelets by the nonionic detergent Nonidet P-40 gave a single radioactive peak at 150,000 MW in sodium dodecyl sulfate-polyacrylamide gel electrophoresis. These findings strongly suggest that the antigenic determinant reacting with this antibody is absent from platelets of Bernard-Soulier syndrome patients and that the deficient molecule is of 150,000 MW. The role of this molecule in subendothelial adhesion and macromolecular-mediated aggregations is discussed.
从一名患有伯纳德-索利尔综合征患者血清中分离出的一种IgG抗体，可使50名正常受试者富含血小板的血浆中的血小板发生凝集，无论其ABO、KOa、KOb、HLA或PlA1血型如何。该抗体与其他三名伯纳德-索利尔综合征患者的血小板无反应。使用该血清（或纯化的IgG）和用125I标记的可溶性膜抗原进行间接免疫沉淀试验，这些抗原是通过非离子去污剂Nonidet P-40从正常血小板中提取的，在十二烷基硫酸钠-聚丙烯酰胺凝胶电泳中，在150,000 MW处出现一个单一的放射性峰。这些发现有力地表明，伯纳德-索利尔综合征患者的血小板缺乏与该抗体反应的抗原决定簇，且缺陷分子的分子量为150,000 MW。本文讨论了该分子在内皮下黏附和大分子介导的聚集过程中的作用。
