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使用改良电泳技术分析因子VIII相关抗原/血管性血友病因子多聚体结构的复杂性。

Analysis of the complexity of the multimeric structure of factor VIII related antigen/von Willebrand protein using a modified electrophoretic technique.

作者信息

Enayat M S, Hill F G

出版信息

J Clin Pathol. 1983 Aug;36(8):915-9. doi: 10.1136/jcp.36.8.915.

DOI:10.1136/jcp.36.8.915
PMID:6409946
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC498421/
Abstract

A method for multimeric analysis of factor VIII related antigen/von Willebrand protein is described. By modifying an existing method the technique has been simplified and optimised so that the different molecular forms of factor VIII related antigen and their triplet structure can be visualised. Clear differences can be seen between patterns in normals and type IIA von Willebrand's disease variants in that the latter lack high and intermediate multimers and also have a different configuration to the triplet pattern in the lower molecular weight multimers. Differences in triplet pattern can also be seen in different type IIA patients. The advantages and further application of this method for investigating the molecular defect in von Willebrand's disease are discussed.

摘要

本文描述了一种用于因子VIII相关抗原/血管性血友病因子多聚体分析的方法。通过改进现有方法，该技术得以简化和优化，从而能够观察到因子VIII相关抗原的不同分子形式及其三联体结构。正常人和IIA型血管性血友病变体的模式之间存在明显差异，后者缺乏高分子量和中等分子量的多聚体，并且在低分子量多聚体中的三联体模式也具有不同的构型。在不同的IIA型患者中也可以看到三联体模式的差异。本文还讨论了该方法在研究血管性血友病分子缺陷方面的优势和进一步应用。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/078b/498421/975e7b9dbda0/jclinpath00513-0082-c.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/078b/498421/bb383f06974b/jclinpath00513-0081-a.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/078b/498421/55dee32a6c7e/jclinpath00513-0082-a.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/078b/498421/55876e7fa7f4/jclinpath00513-0082-b.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/078b/498421/975e7b9dbda0/jclinpath00513-0082-c.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/078b/498421/bb383f06974b/jclinpath00513-0081-a.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/078b/498421/55dee32a6c7e/jclinpath00513-0082-a.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/078b/498421/55876e7fa7f4/jclinpath00513-0082-b.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/078b/498421/975e7b9dbda0/jclinpath00513-0082-c.jpg

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1
Analysis of the complexity of the multimeric structure of factor VIII related antigen/von Willebrand protein using a modified electrophoretic technique.使用改良电泳技术分析因子VIII相关抗原/血管性血友病因子多聚体结构的复杂性。
J Clin Pathol. 1983 Aug;36(8):915-9. doi: 10.1136/jcp.36.8.915.
2
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引用本文的文献

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本文引用的文献

1
Type IIB von Willebrand's disease: differential clearance of endogenous versus transfused large multimer von willebrand factor.IIB型血管性血友病：内源性与输注的大分子多聚体血管性血友病因子的差异清除
Blood. 1982 Dec;60(6):1453-6.
2
Multimeric structure of factor VIII/von Willebrand factor in von Willebrand's disease.血管性血友病中因子VIII/血管性血友病因子的多聚体结构
J Lab Clin Med. 1980 Apr;95(4):590-602.
3
Multimeric composition of factor VIII/von Willebrand factor following administration of DDAVP: implications for pathophysiology and therapy of von Willebrand's disease subtypes.
使用市售增强化学发光试剂盒分析血管性血友病因子多聚体。
J Clin Pathol. 1993 May;46(5):470-3. doi: 10.1136/jcp.46.5.470.
4
Abnormalities of factor VIII related protein multimers in the haemolytic uraemic syndrome.溶血性尿毒症综合征中凝血因子VIII相关蛋白多聚体的异常
Arch Dis Child. 1984 Dec;59(12):1135-40. doi: 10.1136/adc.59.12.1135.
5
Simultaneous inheritance and expression of classical haemophilia A and type IIA von Willebrand's disease.经典型血友病A与IIA型血管性血友病的同时遗传与表达。
J Clin Pathol. 1984 Sep;37(9):1035-9. doi: 10.1136/jcp.37.9.1035.
6
Lack of correlation between factor VIII related antigen multimeric analysis pattern and parallel or non-parallel dose response curves in an ELISA factor VIII related antigen assay.在ELISA法检测因子VIII相关抗原试验中，因子VIII相关抗原多聚体分析模式与平行或非平行剂量反应曲线之间缺乏相关性。
J Clin Pathol. 1984 Feb;37(2):194-9. doi: 10.1136/jcp.37.2.194.
7
Investigation of a kindred with a new autosomal dominantly inherited variant type von Willebrand's disease (possible type IID).对一个患有新型常染色体显性遗传变异型血管性血友病（可能为IID型）的家族进行调查。
J Clin Pathol. 1985 Jun;38(6):665-70. doi: 10.1136/jcp.38.6.665.
8
Changes in the factor VIII complex in diabetic ketoacidosis: evidence of endothelial cell damage?糖尿病酮症酸中毒时凝血因子 VIII 复合物的变化：内皮细胞损伤的证据？
Diabetologia. 1987 Mar;30(3):160-5. doi: 10.1007/BF00274221.
9
In vitro correction of the abnormal multimeric structure of von Willebrand factor in type IIa von Willebrand's disease.IIa型血管性血友病中血管性血友病因子异常多聚体结构的体外校正
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10
Acquired von Willebrand's disease and hypothyroidism: report of a case presenting with menorrhagia.获得性血管性血友病综合征与甲状腺功能减退症：一例以月经过多为表现的病例报告。
Postgrad Med J. 1990 Jun;66(776):474-6. doi: 10.1136/pgmj.66.776.474.
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4
Classification of variant von Willebrand's disease subtypes by analysis of functional characteristics and multimeric composition of factor VIII/von Willebrand factor.通过分析因子VIII/血管性血友病因子的功能特性和多聚体组成对血管性血友病变异亚型进行分类
Ann N Y Acad Sci. 1981;370:205-9. doi: 10.1111/j.1749-6632.1981.tb29733.x.
5
The complex multimeric composition of factor VIII/von Willebrand factor.因子VIII/血管性血友病因子的复杂多聚体组成。
Blood. 1981 Jun;57(6):1140-3.
6
Variant von Willebrand's disease: characterization of two subtypes by analysis of multimeric composition of factor VIII/von Willebrand factor in plasma and platelets.血管性血友病：通过分析血浆和血小板中因子VIII/血管性血友病因子的多聚体组成来鉴定两种亚型
J Clin Invest. 1980 Jun;65(6):1318-25. doi: 10.1172/JCI109795.
7
Factor VIII-related protein circulates in normal human plasma as high molecular weight multimers.VIII因子相关蛋白以高分子量多聚体形式在正常人血浆中循环。
Blood. 1980 Jun;55(6):1056-9.
8
Heightened interaction between platelets and factor VIII/von Willebrand factor in a new subtype of von Willebrand's disease.血管性血友病新亚型中血小板与凝血因子VIII/血管性血友病因子之间的相互作用增强。
N Engl J Med. 1980 May 8;302(19):1047-51. doi: 10.1056/NEJM198005083021902.
9
Abnormal antigenic reactivity of factor VIII/von Willebrand factor subunit in variants of von Willebrand's disease.血管性血友病因子VIII/von Willebrand因子亚基在血管性血友病变异体中的异常抗原反应性。
J Lab Clin Med. 1982 Apr;99(4):481-94.
10
Structural analysis of factor VIII antigen in von Willebrand disease.血管性血友病因子VIII抗原的结构分析
Proc Natl Acad Sci U S A. 1980 Nov;77(11):6832-6. doi: 10.1073/pnas.77.11.6832.