Congenital abnormal plasminogen, Frankfurt I, a cause for recurrent venous thrombosis.
A new abnormal plasminogen, Frankfurt I, has been identified in the plasma of a 42 year-old male patients who had recurring thromboses, thrombophlebitis and pulmonary embolism since his age of 29. Reduced functional and also slightly reduced antigen plasminogen concentrations were found in both the proposituts and his mother. Plasmin generation rates carried out by Streptokinase and Urokinase were also abnormal. The plasmin generated was very unstable in the absence of stabilizing ligands and/or substrates. Crossed immunoelectrophoresis of the purified Frankfurt I revealed a peak with normal size and shape, but displaced with respect to normal Glu-plasminogen toward the anode. Isoelectric focusing followed by zymography on an agarose-fibrin plate proved this observation but did not indicate a separation of the normal from the abnormal plasminogen molecular species, also, fewer bands were found in the abnormal plasminogen isozyme pattern. Kinetic studies of Frankfurt I Glu-plasminogen and plasmin showed that most of the functional abnormality is related to absence of active sites in half of the molecules.
Scharrer
I
I
Dept. of Internal Medicine, University Hospital, Frankfurt/Main, FRG.
Hach-Wunderle
V
V
Wohl
R C
RC
Sinio
L
L
Boreisha
I
I
Robbins
K C
KC
D000328:Adult; D000581:Amidohydrolases; D004789:Enzyme Activation; D005341:Fibrinolysin / Q000378:metabolism; D005858:Germany; D006801:Humans; D007123:Immunoelectrophoresis, Two-Dimensional; D007525:Isoelectric Focusing; D007700:Kinetics; D008297:Male; D010958:Plasminogen / Q000302:isolation & purification* / Q000378:metabolism; D012008:Recurrence; D013924:Thrombophlebitis / Q000097:blood* / Q000151:congenital / Q000209:etiology
D010958:Plasminogen; D005341:Fibrinolysin; D000581:Amidohydrolases; C019665:amidase