| High Molecular Weight Kininogen |
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Information AboutHigh Molecular Weight Kininogen |
| CATEGORIES ABOUT HIGH-MOLECULAR WEIGHT KININOGEN | |
| coagulation system | |
| kinin system | |
| cofactors | |
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NOMENCLATURE High-molecular weight kininogen is one of the Kininogen s, a class of proteins. As with many other coagulation proteins, the protein is named after the patients in whom deficiency was first observed. When the clinical data were combined, it turned out that all patients in fact had a deficiency of the same protein. PHYSIOLOGY HMWK is one of the early participants of the ''intrinsic pathway'' of coagulation, together with Factor XII (Hageman factor) and Prekallikrein . It is 626 Amino Acid s long, and weighs 88 to 120 KD (dependent on Glycosylation ). The kinigogen is not enzymatically active, and only functions as a cofactor for the activation of Kallikrein and Hageman factor. It is also necessary for the activation of factor XI by factor XIIa. The Histidine -rich region (amino acids 420 to 510) participates most strongly in coagulation. HMWK is also a precursor of Bradykinin ; this Vasodilator substance is released through positive feedback by Kallikrein . GENETICS The Gene for both types of Kininogen s is located on the 3rd Chromosome (3q27). MEASUREMENT Measurement of HMWK is usually done with mixing studies, where plasma deficient in HMWK is mixed with the patient's sample and the PTT determined. Results are expressed in % of normal - values under 60% or over 140% are abnormal. ROLE IN DISEASE The existence of HMWK was hypothesised in the 1970s when several patients were described with a deficiency of a class of Plasma protein and a prolonged Bleeding Time and Partial Thromboplastin Time (PTT). Like in deficiency of the associated Hageman Factor , there is no increased risk of Bleeding . EXTERNAL LINK |
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