凝血因子VIII/血管性血友病因子蛋白。半乳糖是血管性血友病因子活性的一个隐蔽决定因素。
Factor VIII/von Willebrand factor protein. Galactose a cryptic determinant of von Willebrand factor activity.
作者信息
Gralnick H R
出版信息
J Clin Invest. 1978 Aug;62(2):496-9. doi: 10.1172/JCI109152.
Abstract
The normal Factor VIII/von Willebrand factor protein has the ability to agglutinate or aggregate normal platelets in the presence of ristocetin (von Willebrand factor activity). Removal of greater than 95% of the sialic acid from this protein by neuraminidase did not affect the von Willebrand factor or procoagulant activity. However, oxidation of the penultimate galactose of the asialo Factor VIII/von Willebrand factor protein with galactose oxidase resulted in a progressive loss of von Willebrand factor activity with no effect on procoagulant activity. Reduction of the 6-aldehydo intermediate by potassium borohydride caused full regeneration of von Willebrand factor activity. These studies confirm the identification of the intact penultimate galactose moiety as a critical determinant of von Willebrand factor activity.
摘要
正常的凝血因子VIII/血管性血友病因子蛋白在瑞斯托霉素存在的情况下具有使正常血小板凝集或聚集的能力（血管性血友病因子活性）。用神经氨酸酶从该蛋白中去除超过95%的唾液酸不会影响血管性血友病因子或促凝血活性。然而，用半乳糖氧化酶氧化去唾液酸凝血因子VIII/血管性血友病因子蛋白的倒数第二个半乳糖会导致血管性血友病因子活性逐渐丧失，而对促凝血活性没有影响。用硼氢化钾还原6-醛中间体可使血管性血友病因子活性完全恢复。这些研究证实了完整的倒数第二个半乳糖部分是血管性血友病因子活性的关键决定因素。
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本文引用的文献
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