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| Assay Range | 31.25 - 2000 pg/ml |
Kininogen-1 (KNG1), also known as alpha-2-thiol proteinase inhibitor,Williams-Fitzgerald-Flaujeac factor or the HMWK-kallikrein factor, is a secreted protein which contains three cystatin domains. Kininogen-1 is a constituent of the blood coagulation system as well as the kinin-kallikrein system. Defects in KNG1 are the cause of high molecular weight kininogen deficiency (HMWK deficiency) which is an autosomal recessive coagulation defect. Patients with HWMK deficiency do not have a hemorrhagic tendency, but they exhibit abnormal surface-mediated activation of fibrinolysis.
