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Fujiwara trait: the first case of kininogen deficiency in Japan.

作者信息

Oh-Ishi S, Ueno A, Uchida Y, Katori M, Hayashi H, Koya H, Kitajima K, Kimura I

出版信息

Adv Exp Med Biol. 1979;120B:93-9.

PMID:517263
Abstract

Asymptomatic identical twins were found to show the prolonged activated partial thromboplastin time, which was corrected by addition of normal, Hageman factor deficient or Fletcher trait plasma but not corrected by Fitzgerald or Williams plasma. The prolonged activated partial thromboplastin time was also corrected by addition of highly purified bovine high molecular weight kininogen but not by low molecular weight kininogen. When total kininogen was measured as the amount of bradykinin released by trypsin on acid treated plasma, only trace amount was detected in Fujiwara and Williams plasmas, although Fitzgerald plasma showed approximately 50% of the total kininogen of normal plasma level. Acetone-kaolin activated amidase activity of plasma kallikrein was not generated by Fujiwara plasma. Substitution with normal plasma in various ratios showed plasma kallikrein activity proportionally to the normal plasma contents. Extrapolation with the values at 120 min after activation gave the prekallikrein content of Fujiwara plasma as 30% of the normal value.

摘要

相似文献

1
Fujiwara trait: the first case of kininogen deficiency in Japan.
Adv Exp Med Biol. 1979;120B:93-9.
2
Abnormalities in the contact activation through factor XII in Fujiwara trait: a deficiency in both high and low molecular weight kininogens with low level of prekallikrein.藤原特质中通过因子 XII 的接触激活异常:高分子量和低分子量激肽原均缺乏,且前激肽释放酶水平较低。
Tohoku J Exp Med. 1981 Jan;133(1):67-80. doi: 10.1620/tjem.133.67.
3
Cleavage of human high molecular weight kininogen markedly enhances its coagulant activity. Evidence that this molecule exists as a procofactor.人高分子量激肽原的裂解显著增强其凝血活性。该分子作为前辅因子存在的证据。
J Clin Invest. 1984 Apr;73(4):954-62. doi: 10.1172/JCI111319.
4
Interactions among Hageman factor (HG, Factor XII), plasma thromboplastin antecedent (PTA, Factor XI), plasma prekallikrein (PK, Fletcher factor) and high molecular weight kininogen (HMW-K, Fitzgerald factor) in blood coagulation.血液凝固过程中,接触因子(HG,凝血因子XII)、血浆凝血活酶前体(PTA,凝血因子XI)、血浆前激肽释放酶(PK,弗莱彻因子)和高分子量激肽原(HMW-K,菲茨杰拉德因子)之间的相互作用。
Adv Exp Med Biol. 1979;120B:61-70.
5
The first cases of Fitzgerald factor deficiency in the Orient: three cases in one family.东方首例菲茨杰拉德因子缺乏症:一个家族中的三例病例。
Acta Haematol. 1980;63(2):107-13. doi: 10.1159/000207379.
6
Severe Fletcher factor (plasma prekallikrein) deficiency with partial deficiency of Hageman factor (factor XII): report of a case with observation on in vivo and in vitro leukocyte chemotaxis.重度弗莱彻因子(血浆前激肽释放酶)缺乏伴哈格曼因子(因子Ⅻ)部分缺乏:1例报告及体内和体外白细胞趋化性观察
Am J Hematol. 1982 May;12(3):261-70. doi: 10.1002/ajh.2830120308.
7
Kininogen deficiency in Fitzgerald trait: role of high molecular weight kininogen in clotting and fibrinolysis.菲茨杰拉德性状中的激肽原缺乏:高分子量激肽原在凝血和纤维蛋白溶解中的作用。
J Lab Clin Med. 1976 Feb;87(2):327-37.
8
Williams trait. Human kininogen deficiency with diminished levels of plasminogen proactivator and prekallikrein associated with abnormalities of the Hageman factor-dependent pathways.威廉姆斯特征。人类激肽原缺乏,纤溶酶原激活物和前激肽释放酶水平降低,与哈格曼因子依赖性途径异常相关。
J Clin Invest. 1975 Dec;56(6):1650-62. doi: 10.1172/JCI108247.
9
Role of high-molecular-weight kininogen in surface-binding and activation of coagulation Factor XI and prekallikrein.高分子量激肽原在凝血因子XI和前激肽释放酶的表面结合及激活中的作用。
Proc Natl Acad Sci U S A. 1977 Oct;74(10):4636-40. doi: 10.1073/pnas.74.10.4636.
10
Purification of high molecular weight kininogen and the role of this agent in blood coagulation.高分子量激肽原的纯化及其在血液凝固中的作用。
J Clin Invest. 1977 Sep;60(3):584-94. doi: 10.1172/JCI108810.