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人类前激肽释放酶缺乏症。

Prekallikrein deficiency in man.

作者信息

Wuepper K D

出版信息

J Exp Med. 1973 Dec 1;138(6):1345-55. doi: 10.1084/jem.138.6.1345.

DOI:10.1084/jem.138.6.1345
PMID:4762550
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC2139450/
Abstract

Blood plasma obtained from an individual with abnormal thromboplastin formation, due to deficiency of Fletcher factor, was fully corrected by 2% of normal, Hageman factor- or PTA-deficient plasma. It was also reconstituted by addition of highly purified human or rabbit prekallikrein. The plasma failed to generate kinin upon exposure to kaolin, a defect which was also corrected by addition of prekallikrein. Prekallikrein antigen was not detectable in this plasma. Fletcher factor-deficient plasma did not support the normal generation of PF/dil when dilute plasma was incubated in glass vessels and injected intracutaneously. Small quantities of Fletcher factor-deficient or Hageman factor-deficient plasma corrected the ability of the other to generate PF/dil. The formation of plasmin in dilute, acidified plasma incubated with kaolin was also abnormal in Fletcher factor-deficient plasma. Plasmin generation was normalized by addition of prekallikrein or small quantities of Hageman factor-deficient plasma. The data support the identity of Fletcher factor and prekallikrein.

摘要

从因弗勒彻因子缺乏而导致凝血活酶生成异常的个体获取的血浆,可被2%的正常血浆、哈格曼因子或血浆促凝血酶原激酶(PTA)缺乏的血浆完全纠正。通过添加高度纯化的人或兔前激肽释放酶也可使其恢复。该血浆在接触高岭土时无法生成激肽,添加前激肽释放酶也可纠正这一缺陷。在此血浆中检测不到前激肽释放酶抗原。当稀释血浆在玻璃容器中孵育并皮内注射时,弗勒彻因子缺乏的血浆不支持正常生成PF/dil。少量弗勒彻因子缺乏或哈格曼因子缺乏的血浆可纠正另一方生成PF/dil的能力。在与高岭土孵育的稀释酸化血浆中,弗勒彻因子缺乏的血浆中纤溶酶的形成也异常。通过添加前激肽释放酶或少量哈格曼因子缺乏的血浆可使纤溶酶生成正常化。这些数据支持弗勒彻因子与前激肽释放酶的一致性。

相似文献

1
Prekallikrein deficiency in man.人类前激肽释放酶缺乏症。
J Exp Med. 1973 Dec 1;138(6):1345-55. doi: 10.1084/jem.138.6.1345.
2
Fletcher factor deficiency. A diminished rate of Hageman factor activation caused by absence of prekallikrein with abnormalities of coagulation, fibrinolysis, chemotactic activity, and kinin generation.弗莱彻因子缺乏症。由于缺乏前激肽释放酶导致哈格曼因子激活速率降低,伴有凝血、纤维蛋白溶解、趋化活性和激肽生成异常。
J Clin Invest. 1974 Feb;53(2):622-33. doi: 10.1172/JCI107597.
3
Clinical and physiologic studies of two siblings with prekallikrein (Fletcher factor) deficiency.两名前激肽释放酶(弗莱彻因子)缺乏症同胞的临床和生理学研究。
Am J Med. 1976 May 10;60(5):654-64. doi: 10.1016/0002-9343(76)90500-3.
4
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.
5
Fletcher factor deficiency (Report of a new case).弗莱彻因子缺乏症(一例新病例报告)
Ric Clin Lab. 1983 Oct-Dec;13(4):443-8.
6
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.
7
Prekallikrein (Fletcher factor) deficiency.前激肽释放酶(弗莱彻因子)缺乏症。
Ann Clin Lab Sci. 1985 Jul-Aug;15(4):279-85.
8
Immunoelectrophoretic studies of prekallikrein in human plasma.人血浆中前激肽释放酶的免疫电泳研究。
Thromb Res. 1982 Feb 1;25(3):267-75. doi: 10.1016/0049-3848(82)90246-8.
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Deficiency of factor XII-dependent plasminogen proactivator in prekallikrein-deficient plasma.前激肽释放酶缺乏血浆中依赖因子XII的纤溶酶原激活剂缺乏。
J Lab Clin Med. 1978 Jan;91(1):148-55.
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Initiation of plasma prorenin activation by Hageman factor-dependent conversion of plasma prekallikrein to kallikrein.通过Hageman因子依赖性地将血浆前激肽释放酶转化为激肽释放酶来启动血浆肾素原激活。
Proc Natl Acad Sci U S A. 1979 Nov;76(11):5914-8. doi: 10.1073/pnas.76.11.5914.

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本文引用的文献

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A familial hemorrhagic trait associated with a deficiency of a clot-promoting fraction of plasma.一种与血浆中促凝成分缺乏相关的家族性出血性状。
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THE INDUCTION OF PERMEABILITY-INCREASING ACTIVITY IN HUMAN PLASMA BY ACTIVATED HAGEMAN FACTOR.活化的哈格曼因子诱导人血浆中通透性增加活性
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Activation of plasma by contact with glass: evidence for a common reaction which releases plasma kinin and initiates coagulation.血浆与玻璃接触后的激活:释放血浆激肽并启动凝血的共同反应的证据。
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Evidence for a new plasma thromboplastin factor. I. Case report, coagulation studies and physicochemical properties.一种新的血浆凝血活酶因子的证据。I. 病例报告、凝血研究及物理化学性质。
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The relation of 'Fletcher Factor' to factors XI and XII.“弗莱彻因子”与因子XI和因子XII的关系。
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Effect of plasma kallikrein on coagulation in vitro.血浆激肽释放酶对体外凝血的影响。
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Plasma prekallikrein: isolation, characterization, and mechanism of activation.血浆前激肽释放酶:分离、特性及激活机制
J Exp Med. 1972 Jan;135(1):1-20. doi: 10.1084/jem.135.1.1.
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The fibrinolytic pathway of human plasma. Isolation and characterization of the plasminogen proactivator.人血浆的纤维蛋白溶解途径。纤溶酶原激活剂的分离与特性鉴定。
J Exp Med. 1972 Dec 1;136(6):1378-93. doi: 10.1084/jem.136.6.1378.
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Studies on a complex mechanism for the activation of plasminogen by kaolin and by chloroform: the participation of Hageman factor and additional cofactors.高岭土和氯仿激活纤溶酶原的复杂机制研究:哈格曼因子及其他辅助因子的作用
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