Figure 3.
Clearance kinetics of p.(Cys1084Tyr)-VWF. Time course of the postdesmopressin response in siblings III-1 (A) and III-2 (B). Markedly reduced VWF:Ag, VWF:GPIbM and FVIII:C half-lives were observed following the 1-hour peak. The dashed line represents normalized VWF:Ag, VWF:GPIbM and FVIII:C plasma levels (0.5 IU/mL). (C) The influence of p.(Cys1084Tyr) on VWF clearance in a mouse model of VWD. Prolonged survival of p.(Cys1084Tyr)-VWF was observed. Data were fitted to a 1-phase exponential decay (n = 3 mice per time point).

Clearance kinetics of p.(Cys1084Tyr)-VWF. Time course of the postdesmopressin response in siblings III-1 (A) and III-2 (B). Markedly reduced VWF:Ag, VWF:GPIbM and FVIII:C half-lives were observed following the 1-hour peak. The dashed line represents normalized VWF:Ag, VWF:GPIbM and FVIII:C plasma levels (0.5 IU/mL). (C) The influence of p.(Cys1084Tyr) on VWF clearance in a mouse model of VWD. Prolonged survival of p.(Cys1084Tyr)-VWF was observed. Data were fitted to a 1-phase exponential decay (n = 3 mice per time point).

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