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Patients lacking high molecular weight (HMW) kininogen have profound abnormalities of the Hageman factor-dependent pathways of coagulation, kinin formation, and fibrinolysis. The ability of HMW kininogen to potentiate the Hageman factor fragments (HFf) activation of prekallikrein and Factor XI in plasma was studied. HFf only partially converted Factor XI to XIa and prekallikrein to kallikrein in plasma deficient in HMW kininogen (Williams trait), while enhanced activation of Factor XI and prekallikrein by HFf resulted after reconstitution with HMW kininogen. In a system using highly purified components, HMW kininogen increased the initial rate of prekallikrein activation whether the kallikrein formed was assayed by arginine esterase activity or kininforming ability. The potentiation of prekallikrein activation occurred over a 12-fold range of enzyme (HFf) concentration and was nonhyperbolic with respect to substrate (prekallikrein). HMW kininogen exerted its effect even in the absence of prekallikrein since the hydrolysis of acetylglycyl-lysine methyl ester by HFf was increased by HMW kininogen. These results suggest that one of the functions of HMW kininogen is to augment the catalytic action of HFf.
C Y Liu, and
C F Scott, and
A Bagdasarian, and
J V Pierce, and
A P Kaplan, and
R W Colman
November 1976,
Thrombosis research,
C Y Liu, and
C F Scott, and
A Bagdasarian, and
J V Pierce, and
A P Kaplan, and
R W Colman
July 1977,
The Journal of clinical investigation,
C Y Liu, and
C F Scott, and
A Bagdasarian, and
J V Pierce, and
A P Kaplan, and
R W Colman
August 1980,
The Journal of laboratory and clinical medicine,
C Y Liu, and
C F Scott, and
A Bagdasarian, and
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A P Kaplan, and
R W Colman
September 1986,
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C Y Liu, and
C F Scott, and
A Bagdasarian, and
J V Pierce, and
A P Kaplan, and
R W Colman
January 1979,
Thrombosis research,
C Y Liu, and
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A Bagdasarian, and
J V Pierce, and
A P Kaplan, and
R W Colman
February 1979,
Proceedings of the National Academy of Sciences of the United States of America,
C Y Liu, and
C F Scott, and
A Bagdasarian, and
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A P Kaplan, and
R W Colman
July 1983,
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C Y Liu, and
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A Bagdasarian, and
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R W Colman
August 1976,
Proceedings of the National Academy of Sciences of the United States of America,
C Y Liu, and
C F Scott, and
A Bagdasarian, and
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A P Kaplan, and
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Agents and actions,
C Y Liu, and
C F Scott, and
A Bagdasarian, and
J V Pierce, and
A P Kaplan, and
R W Colman
April 1980,
The Journal of laboratory and clinical medicine,
