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一种伴有因子VIII/血管性血友病因子蛋白异常的血管性血友病-于尔根斯综合征变体（作者译）

[A variant of the von Willebrand-Jürgens-syndrome with abnormalities of the factor VIII/von Willebrand factor protein (author's transl)].

作者信息

Hasler K, Böttcher D, Engelhardt R

出版信息

Blut. 1979 Jan 22;38(1):25-34. doi: 10.1007/BF01082925.

DOI:10.1007/BF01082925
PMID:310328
Abstract

A family is reported with a variant of von Willebrand's disease. The members of this family showed a qualitative defect of the factor VII/von Willebrand factor protein. The qualitative defect was characterized by an abnormal electrophoretical mobility of factor VIII-related antigen and an abnormal elution pattern as demonstrated by gelfiltration on Sepharose 4 B. Factor VIII-subunits in these patients were found to be normal by polyacrylamidgelelektrophoresis.

摘要

报道了一个患有血管性血友病变异型的家族。该家族成员表现出因子 VIII/血管性血友病因子蛋白的质量缺陷。这种质量缺陷的特征是 VIII 因子相关抗原的异常电泳迁移率以及通过 Sepharose 4 B 凝胶过滤所显示的异常洗脱模式。通过聚丙烯酰胺凝胶电泳发现这些患者的 VIII 因子亚基正常。

相似文献

1
[A variant of the von Willebrand-Jürgens-syndrome with abnormalities of the factor VIII/von Willebrand factor protein (author's transl)].一种伴有因子VIII/血管性血友病因子蛋白异常的血管性血友病-于尔根斯综合征变体（作者译）
Blut. 1979 Jan 22;38(1):25-34. doi: 10.1007/BF01082925.
2
[Von Willebrand's syndrome (author's transl)].[血管性血友病综合征（作者译）]
Nouv Rev Fr Hematol Blood Cells. 1977;18(2):371-82.
3
[Biological diagnosis of von Willebrand's disease (author's transl)].血管性血友病的生物学诊断（作者译）
Sangre (Barc). 1978;23(5B):728-45.
4
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.
5
Von Willebrand's disease: combined qualitative and quantitative abnormalities.血管性血友病：定性和定量异常并存
N Engl J Med. 1977 May 5;296(18):1024-30. doi: 10.1056/NEJM197705052961802.
6
Studies of the human factor VIII/von Willebrand factor protein. III. Qualitative defects in von Willebrand's disease.人凝血因子VIII/血管性血友病因子蛋白的研究。III. 血管性血友病的定性缺陷。
J Clin Invest. 1975 Oct;56(4):814-27. doi: 10.1172/JCI108160.
7
Electrophoretic heterogeneity of normal factor VIII/Von Willebrand protein, and abnormal electrophoretic mobility in patients with Von Willebrand's disease.正常因子VIII/血管性血友病因子蛋白的电泳异质性，以及血管性血友病患者的异常电泳迁移率。
J Lab Clin Med. 1976 Feb;87(2):185-94.
8
[Von Willebrand's disease (author's transl)].[血管性血友病（作者译）]
Rinsho Ketsueki. 1977 May;18(5):575-86.
9
Structure - function relationship of factor VIII/von Willebrand factor. Application to the study of variant von Willebrand's disease and cryosupernatant prepared from normal plasma.因子VIII/血管性血友病因子的结构-功能关系。应用于血管性血友病变异型及正常血浆制备的冷上清液的研究。
Thromb Res. 1980;19(4-5):671-85. doi: 10.1016/0049-3848(80)90039-0.
10
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.

本文引用的文献

1
Protein measurement with the Folin phenol reagent.使用福林酚试剂进行蛋白质测定。
J Biol Chem. 1951 Nov;193(1):265-75.
2
The spectrum of von Willebrand's disease.血管性血友病的谱系
Thromb Diath Haemorrh. 1967 Aug 15;18(1-2):40-56.
3
Willebrand factor and ristocetin. II. Relationship between Willebrand factor, Willebrand antigen and factor VIII activity.血管性血友病因子与瑞斯托霉素。II. 血管性血友病因子、血管性血友病抗原与凝血因子VIII活性之间的关系。
Br J Haematol. 1974 Dec;28(4):579-99. doi: 10.1111/j.1365-2141.1974.tb06676.x.
4
Proceedings: An abnormality of factor-VIII-related protein (FVIIIRP)--possible variants of von Willebrand's disease.会议记录：因子VIII相关蛋白（FVIIIRP）异常——血管性血友病的可能变异型
Br J Haematol. 1974 Sep;28(1):143-4.
5
Two genetic variants of von Willebrand's disease.血管性血友病的两种基因变体。
N Engl J Med. 1973 Mar 22;288(12):595-8. doi: 10.1056/NEJM197303222881202.
6
A variant of factor 8 related antigen.因子8相关抗原的一种变体。
Br J Haematol. 1974 Mar;26(3):435-40. doi: 10.1111/j.1365-2141.1974.tb00484.x.
7
The subunit structure of normal and hemophilic factor VIII.正常及血友病因子VIII的亚基结构
J Clin Invest. 1973 Sep;52(9):2198-210. doi: 10.1172/JCI107405.
8
Glycoprotein staining following electrophoresis on acrylamide gels.丙烯酰胺凝胶电泳后的糖蛋白染色。
Anal Biochem. 1969 Jul;30(1):148-52. doi: 10.1016/0003-2697(69)90383-2.
9
Letter: 8-related antigen and von Willebrand factor.信函：8相关抗原与血管性血友病因子
Lancet. 1974 Apr 27;1(7861):803-4. doi: 10.1016/s0140-6736(74)92867-0.
10
Evidence for a qualitative defect in factor-VIII-related antigen in von Willebrand's disease.血管性血友病中因子 VIII 相关抗原存在质量缺陷的证据。
Lancet. 1974 Apr 6;1(7858):594-6. doi: 10.1016/s0140-6736(74)92652-x.