Reduction of endogenous plasma GlcCer by recombinant glucocerebrosidase is associated with reduced anticoagulant response to APC:protein S.

Following hydrolysis of endogenous plasma GlcCer for 3 minutes due to addition of recombinant glucocerebrosidase to plasma, the anticoagulant response to APC:protein S was immediately assayed, and using parallel aliquots, residual plasma GlcCer levels (right ordinate, ▵) were determined by HPLC analysis as described in “Patients, materials, and methods.” The clotting times (left ordinate) observed in the presence (●) and absence (○) of APC:protein S are shown. The plasma GlcCer level prior to glucocerebrosidase treatment was defined as 100%.