Figure 6
Figure 6. Hydrodynamic mVWF multimer structure. VWF knockout mice expressing wild-type, R1205H, or Y1584C mouse VWF were sampled after hydrodynamic injection and evaluated for multimeric structure. Plasma was run on a 1.4% agarose gel at an mVWF concentration of 1.0 U/mL. (A) Exemplar multimers are shown for (i) wild-type, (ii) R1205H, and (iii) Y1584C. The dotted line represents high molecular weight multimers, greater than 10 multimer bands. Arrowheads indicate the top bands in the enlarged inset bands, with a line through the inset figure illustrating the single band of lower molecular weight in the recombinant proteins compared with C57Bl/6 normal plasma pool (CNP) triplet multimer bands. The asterisk (*) denotes a multimer from a clotted sample. Wild-type day 42 and Y1584C day 28 samples were less than 1 U/mL. (B) Multimer analysis was performed on VWF knockout mice expressing mVWF after hydrodynamic injection by counting the total number of resolved multimer bands in each lane. N ≥ 4. (C) Multimer analysis on VWF knockout mice expressing mVWF and mADAMTS13 after hydrodynamic injection. N ≥ 4.

Hydrodynamic mVWF multimer structure. VWF knockout mice expressing wild-type, R1205H, or Y1584C mouse VWF were sampled after hydrodynamic injection and evaluated for multimeric structure. Plasma was run on a 1.4% agarose gel at an mVWF concentration of 1.0 U/mL. (A) Exemplar multimers are shown for (i) wild-type, (ii) R1205H, and (iii) Y1584C. The dotted line represents high molecular weight multimers, greater than 10 multimer bands. Arrowheads indicate the top bands in the enlarged inset bands, with a line through the inset figure illustrating the single band of lower molecular weight in the recombinant proteins compared with C57Bl/6 normal plasma pool (CNP) triplet multimer bands. The asterisk (*) denotes a multimer from a clotted sample. Wild-type day 42 and Y1584C day 28 samples were less than 1 U/mL. (B) Multimer analysis was performed on VWF knockout mice expressing mVWF after hydrodynamic injection by counting the total number of resolved multimer bands in each lane. N ≥ 4. (C) Multimer analysis on VWF knockout mice expressing mVWF and mADAMTS13 after hydrodynamic injection. N ≥ 4.

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