Williams trait. Human kininogen deficiency with diminished levels of plasminogen proactivator and prekallikrein associated with abnormalities of the Hageman factor-dependent pathways.

@article{Colman1975WilliamsTH,
  title={Williams trait. Human kininogen deficiency with diminished levels of plasminogen proactivator and prekallikrein associated with abnormalities of the Hageman factor-dependent pathways.},
  author={Robert W. Colman and Andranik Bagdasarian and Richard C. Talamo and Cheryl F. Scott and Matthew M. Seavey and Jorge A. Guimar{\~a}es and Jack V. Pierce and Allen P. Kaplan},
  journal={The Journal of clinical investigation},
  year={1975},
  volume={56 6},
  pages={
          1650-62
        }
}
An asymptomatic woman (Ms. Williams) was found to have a severe abnormality in the surface-activated intrinsic coagulation, fibrinolytic, and kinin-generating pathways. Assays for known coagulation factors were nromal while Fletcher factor (pre-kallikrein) was 45%, insufficient to account for the observed markedly prolonged partial thromboplastin time. Plasminogen proactivator was present at 20% of normal levels and addition of highly purified plasminogen proactivator containing 10% plasminogen… Expand
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.
TLDR
Results suggest that Fujiwara trait is very similar to Williams trait in that both plasmas were deficient in HMW and LMW kininogens with reduced content of prekallikrein. Expand
Potentiation of the function of Hageman factor fragments by high molecular weight kininogen.
TLDR
The results suggest that one of the functions of HMW kininogen is to augment the catalytic action of HFf, and that the hydrolysis of acetylglycyl-lysine methyl ester by HFf was increased by HMW Kininogen. Expand
Purification of high molecular weight kininogen and the role of this agent in blood coagulation.
  • H. Saito
  • Chemistry, Medicine
  • The Journal of clinical investigation
  • 1977
TLDR
HMW-kininogen specifically repaired impaired surface-mediated plasma reactions of Fitzgerald trait plasma, but did not affect those of Hageman trait and Fletcher trait plasma. Expand
Plasma contact system, kallikrein-kinin system and antiphospholipid-protein antibodies in thrombosis and pregnancy.
Coagulation factor XII, prekallikrein and high molecular weight kininogen are known as plasma contact proteins in the intrinsic pathway of blood coagulation. Deficiencies of these proteins are notExpand
Hageman-factor-dependent fibrinolysis: generation of fibrinolytic activity by the interaction of human activated factor XI and plasminogen.
TLDR
The Hageman-factor-dependent plasminogen proactivator previously reported to be present in the gamma-globulin fraction of normal human plasma is a function of prekallikrein and factor XI, while the activity observed in prekalicrein-deficient plasma is attributable to factor XI. Expand
Hageman-factor-dependent fibrinolysis: generation of fibrinolytic activity by the interaction of human activated factor XI and plasminogen
TLDR
The Hageman-factor-dependent plasminogen proactivator previously reported to be present in the gamma-globulin fraction of normal human plasma is a function of prekallikrein and factor XI, while the activity observed in prekalicrein-deficient plasma is attributable to factor XI. Expand
First Case of Compound Heterozygous Mutations in the Kininogen Gene Causing Severe High Molecular Weight Kininogen Deficiency
High molecular weight kininogen (HK), prekallikrein (PK) and Factor XII (FXII) form the plasma kallikrein/kinin system (KKS) which has been traditionally understood as the »contact activation system«Expand
Hageman factor substrates. Human plasma prekallikrein: mechanism of activation by Hageman factor and participation in hageman factor-dependent fibrinolysis.
TLDR
The two bands observed are shown to represent prekallikrein by functional, immunochemical, and structural criteria and two factors capable of activating plasminogen to plAsmin have been isolated; one is identified as kallik rein. Expand
Activation of factor XII in human plasma: protection by benzamidine of the cofactor function of high molecular weight kininogen.
TLDR
The cofactor capacity of high molecular weight kininogen (HMrK) was protected against destruction by a serine protease which was not plasma kallikrein, and an average lower level of cofactor-active HMrK in plasma preparations from healthy men and women demonstrated. Expand
Kallikrein-Kinin System in Pathologic Conditions
TLDR
A bimodal distribution of Hageman factor concentrations have been found in study of 50 heterozygotes suggesting that at least two normal alleles control production of Hagman factor. Expand
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References

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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.
TLDR
The ability of prekallikrein to correct the coagulation, fibrinolytic, kinin-generating, and chemotactic defects of Fletcher factor-deficient plasma is consistent with the identity of the Fletcher factor and prekalikre in. Expand
PREKALLIKREIN DEFICIENCY IN MAN
  • K. Wuepper
  • Chemistry, Medicine
  • The Journal of experimental medicine
  • 1973
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. ItExpand
The fibrinolytic pathway of human plasma. Isolation and characterization of the plasminogen proactivator.
The conversion of the plasminogen proactivator to plasminogen activator by activated Hageman factor or its fragments has been recognized as an essential step in the conversion of plasminogen toExpand
A PREALBUMIN ACTIVATOR OF PREKALLIKREIN : II. DERIVATION OF ACTIVATORS OF PREKALLIKREIN FROM ACTIVE HAGEMAN FACTOR BY DIGESTION WITH PLASMIN
Activation of a plasma fraction containing unactivated Hageman factor and prekallikrein followed by chromatography of this fraction on DEAE-cellulose revealed four peaks having bradykinin-generatingExpand
Isolation of two functionally different kininogens from human plasma--separation from proteinase inhibitors and interaction with plasma kallikrein.
TLDR
There was a well defined functional difference between the two kininogens with respect to kinin generation by plasma kallikrein and this enzyme released kinin at a much faster rate from the HMW-Kininogen than from the LMW-kininogen. Expand
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TLDR
It is implied that agents like streptokinase, which convert plasminogen to pl asmin "indirectly" (in two steps) should not be termed plasmineoplastins, but rather lysokinases (1), and the specific term plasminioplastin is proposed for those agents which produce plAsmin by "direct" action on human and animal plasMINogen. Expand
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TLDR
The levels of prekallikrein antigen in plasma samples from patients with severe liver disease remains 40% of normal, while the functional kall Kikrein activity was about 8%. Expand
New Hemophilia-like Disease Caused by Deficiency of a Third Plasma Thromboplastin Factor.∗
TLDR
A new clotting factor, designated as plasma thromboplastin antecedent (PTA) is present in BaSO4-treated normal plasma and normal serum, and its clotting times are only moderately prolonged in patients with mild hemorrhagic disease. Expand
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TLDR
The effect of highly purified inhibitor of the first component of complement, alpha2 macroglobulin, and alpha1 antitrypsin on the components of the fibrinolytic pathway in human plasma has been examined and all three plasma inhibitors were active against plasmin. Expand
The Purification and Some Properties of Two Different Kallidinogens from Human Plasma
Although bovine kininogen has been highly purified (Habermann et al., 1963; Suzuki et al, 1965), very little has been published on the purification of human plasma kallidinogen (Webster and Pierce,Expand
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