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各种凝血因子 VIII 浓缩物中凝血因子 VIII 蛋白和凝血因子 XIII 的特性

Characteristics of the factor VIII protein and Factor XIII in various factor VIII concentrates.

作者信息

Nilsson I M, Holmberg L, Stenberg P, Henriksson P

出版信息

Scand J Haematol. 1980 Apr;24(4):340-9. doi: 10.1111/j.1600-0609.1980.tb01593.x.

DOI:10.1111/j.1600-0609.1980.tb01593.x
PMID:6774412
Abstract

The in vitro properties of 5 factor VIII preparations (AHF-Kabi, Hemofil Hyland, AHF-Profilate Abbott, Kryobulin Immuno and Factorate High Purity Armour) and an ordinary cryoprecipitate were studied with reference to factor VIII clotting activity (VIII:C), factor VIII clotting antigen (VIII:CAg), factor VIII related antigen (VIIIR:Ag) (EI, IRMA, CIE), ristocetin cofactor activity (VIIIR:RCF), fibrinogen and factor XIII. All the preparations with the exception of Factorate had higher levels of VIII:CAg than VIII:C indicating inactivation of the biological activity of VIII:C during the procedure. AHF-Kabi (fraction I-0) and the cryoprecipitate, the only preparations capable of normalising the defect in patients with von Willebrand's disease, showed the same level of VIIIR:Ag determined by EI and by IRMA, while all the other preparations (i.e. cryoprecipitates purified further in different ways) had considerably lower levels of VIIIR:Ag determined by IRMA than by EI. Based on these in vitro techniques it seems to be possible to predict which preparations can be used successfully in patients with von Willebrand's disease, while no such conclusions can be made from VIIIR:RCF determinations. EI yielded similar concentrations of factor XIII a subunit in all the preparations tested. 3 functional assays showed high factor XIII activities in AHF-Kabi but low or no activities in the others. Thus, considerable differences were found on the in vitro properties of the proteins in 5 factor VIII concentrates and a cryoprecipitate. The action of proteases and the techniques used in the purification procedure are probably of crucial importance for the properties of the various factors.

摘要

对5种凝血因子VIII制剂(卡比凝血因子VIII、海兰血纤维蛋白溶酶、雅培凝血因子VIII浓缩剂、免疫冷沉淀球蛋白和高纯度阿莫尔凝血因子VIII)以及一种普通冷沉淀的体外特性进行了研究,涉及凝血因子VIII凝血活性(VIII:C)、凝血因子VIII凝血抗原(VIII:CAg)、凝血因子VIII相关抗原(VIIIR:Ag)(酶免疫测定法、免疫放射测定法、免疫电泳法)、瑞斯托霉素辅因子活性(VIIIR:RCF)、纤维蛋白原和凝血因子XIII。除凝血因子VIII外,所有制剂的VIII:CAg水平均高于VIII:C,表明在制备过程中VIII:C的生物活性失活。卡比凝血因子VIII(I-0组分)和冷沉淀是仅有的能够使血管性血友病患者的缺陷恢复正常的制剂,通过酶免疫测定法和免疫放射测定法测定的VIIIR:Ag水平相同,而所有其他制剂(即以不同方式进一步纯化的冷沉淀)通过免疫放射测定法测定的VIIIR:Ag水平比通过酶免疫测定法测定的水平低得多。基于这些体外技术,似乎有可能预测哪些制剂可成功用于血管性血友病患者,而从VIIIR:RCF测定结果无法得出此类结论。酶免疫测定法在所有测试制剂中测得的凝血因子XIII a亚基浓度相似。3种功能测定法显示卡比凝血因子VIII中的凝血因子XIII活性高,而其他制剂中的活性低或无活性。因此,发现5种凝血因子VIII浓缩剂和一种冷沉淀中蛋白质的体外特性存在相当大的差异。蛋白酶的作用以及纯化过程中使用的技术可能对各种因子的特性至关重要。

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