Arginine carboxypeptidase (CPR) is a single-chain plasma protein generated
during coagulation from a precursor (proCPR), proCPR is the same molecule a
s thrombin activable fibrinolysis inhibitor (TAFI), which retards fibrin cl
ot lysis in vitro and most likely modulates fibrinolysis in vivo. In this s
tudy, the amount of CPR-total, which includes proCPR (TAFI) and CPR (activa
ted TAFI), in hemophiliac patients was evaluated using a newly developed en
zyme linked immunosorbent assay (ELISA). The amount of CPR-total in plasma
or serum of most of the hemophiliac patients was in the range of healthy in
dividuals. There was no significant difference in hemophiliac patients with
or without HIV-1 infection. However two out of the 74 hemophiliac patients
shelved a significantly high level. The upregulation of CPR-total might co
ntribute to compensate for inefficient coagulation in some hemophiliac indi
viduals.