Figure 2
Plasma and whole blood fibrinolysis. (A-C) Turbidity measured in triplicate at 405 nm in plasma from TM-AC cases I.1 and II.1 (n = 6), high-TM model plasma containing 500 ng/mL sTM (n = 3), or control plasma without added sTM (n = 3) diluted 30:70 in TBST buffer [10 mM tris(hydroxymethyl)aminomethane, 140 nM NaCl, 0.01% Tween-20, pH7.4] containing 16 mM phospholipids. Clotting was initiated with 0.1 U/mL thrombin and 10.6 mM CaCl2 in the presence of 300 pM recombinant human tissue plasminogen activator. The curves represent mean turbidity with no carboxypeptidase inhibitor (no CPI), with 65 μg/mL MA-T12D11 or 25 μg/mL PTCI. (D) Time to 50% clot lysis in the TM-AC (n = 6), high-TM model (n = 3), and control (n = 3) plasma samples in the absence or presence of 65 μg/mL MA-T12D11 or 25 μg/mL PTCI. (E) Representative ROTEM viscoelastometry traces following clot formation with the EXTEM reagent in whole blood from a healthy control and from TM-AC case II.2. Traces are shown in the absence and presence of 5 μg/mL PTCI. (F) Time to 90% clot lysis in TM-AC (n = 6), high-TM model (n = 3), and healthy control (n = 20) blood in the absence or presence of 1 to 10 μg/mL PTCI. (G) Time to 90% clot lysis in high-TM model blood samples in the absence or presence of 25 to 100 μg/mL rFVIIa or 0.25 to 0.75 U/mL aPCC (n = 3). Data are means ± SEM; **P ≤ .01. NS, not significant.

Plasma and whole blood fibrinolysis. (A-C) Turbidity measured in triplicate at 405 nm in plasma from TM-AC cases I.1 and II.1 (n = 6), high-TM model plasma containing 500 ng/mL sTM (n = 3), or control plasma without added sTM (n = 3) diluted 30:70 in TBST buffer [10 mM tris(hydroxymethyl)aminomethane, 140 nM NaCl, 0.01% Tween-20, pH7.4] containing 16 mM phospholipids. Clotting was initiated with 0.1 U/mL thrombin and 10.6 mM CaCl2 in the presence of 300 pM recombinant human tissue plasminogen activator. The curves represent mean turbidity with no carboxypeptidase inhibitor (no CPI), with 65 μg/mL MA-T12D11 or 25 μg/mL PTCI. (D) Time to 50% clot lysis in the TM-AC (n = 6), high-TM model (n = 3), and control (n = 3) plasma samples in the absence or presence of 65 μg/mL MA-T12D11 or 25 μg/mL PTCI. (E) Representative ROTEM viscoelastometry traces following clot formation with the EXTEM reagent in whole blood from a healthy control and from TM-AC case II.2. Traces are shown in the absence and presence of 5 μg/mL PTCI. (F) Time to 90% clot lysis in TM-AC (n = 6), high-TM model (n = 3), and healthy control (n = 20) blood in the absence or presence of 1 to 10 μg/mL PTCI. (G) Time to 90% clot lysis in high-TM model blood samples in the absence or presence of 25 to 100 μg/mL rFVIIa or 0.25 to 0.75 U/mL aPCC (n = 3). Data are means ± SEM; **P ≤ .01. NS, not significant.

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