Prekallikrein deficiency in a kindred with kininogen deficiency and Fitzgerald trait clotting defect. Evidence that high molecular weight kininogen and prekallikrein exist as a complex in normal human plasma.
Plasma from an individual with a hereditary deficiency of kininogens is deficient in kininogen antigens; heterozygous relatives are partially deficient in plasma kininogen antigens. In addition, plasma from the proband is partially deficient in functional and antigenic properties of a plasma prekallikrein, and the relatives heterozygous for kininogen deficiency are also partially deficient in the plasma prekallikrein. It is possible that the defects are both inherited and that the inheritance of a deficiency of prekallikrein is genetically linked to the inheritance of a deficiency of kininogen. Alternatively, it is possible that the deficiency of prekallikrein may be due to its hypercatabolism which could be a consequence of a deficiency of high molecular weight kininogen that may stabilize the prekallikrein in plasma. Evidence to support this possibility is presented by the fact that prekallikrein and high molecular weight kininogen apparently exist as a complex in normal plasma, because monospecific antiserum to kininogen removed both high molecular weight kininogen and prekallikrein from plasma, and vice versa. Moreover, prekallikrein was not adsorbed from kininogen-deficient plasma by antiserum to kininogen unless high molecular weight kininogen was first added to the plasma. Low molecular weight kininogen did not participate in these reactions.
D001777:Blood Coagulation; D001778:Blood Coagulation Disorders / Q000097:blood* / Q000235:genetics; D005260:Female; D006801:Humans; D007106:Immune Sera; D007122:Immunoelectrophoresis; D066298:In Vitro Techniques; D007610:Kallikreins / Q000172:deficiency*; D007704:Kininogens / Q000032:analysis / Q000097:blood*; D008297:Male; D010375:Pedigree; D011288:Prekallikrein / Q000032:analysis / Q000172:deficiency*
D007106:Immune Sera; D007704:Kininogens; D011288:Prekallikrein; D007610:Kallikreins