Fibrinogen Pamplona. A new molecular variant
Gastearena, J.; Rocha, E.; Orué, M.T.; Narvaiza, M.J.; Perez Equiza, E.; Fernández, J.
Sangre 26(5-A): 575-587
1981
ISSN/ISBN: 0036-4355 PMID: 7323915 Document Number: 177161
An abnormal fibrinogen was found in a 39 yr old woman. The assessment of prothrombin time, thrombin time, activated partial thromboplastin time and reptilase time showed impaired values. Thrombin time was partially corrected by addition of normal plasma and totally by addition of protamin sulfate. When the patient's purified fibrinogen was added to a control plasma, the thrombin time was strikingly prolonged. The family survey disclosed the same anomalies in 2 of 4 children studied. The analysis of the purified fibrinogen molecule showed normal results of the fibrinopeptide release, fibrin stabilization, sedimentation quotient, hexosamine contents and SDS-polyacrylamide gel electrophoresis. Increasing human and bovine thrombin concentrations, as well as pH and ionic strength variations, failed to dot the purified molecule. When compared with normal, the purified molecule showed antigenic identity, although both immunoelectrophoresis and bidimensional electrophoresis showed slight cathodic mobility. Monomer aggregation was impaired. The sialic acid content was abnormally high, 0.068 .mu.g/ml (normal 0.038 .mu.g/mg), and the coagulability index was 13.8% (normal 96 .+-. 2%). The native fibrinogen thrombin time (> 120 s) was shortened to 32 s after dialyzing the molecule.