Figure 2
Figure 2. Wild-type and mutant VWF expression in cellular models. (A) Western blot analysis of wild-type and mutant VWF in conditioned media (top panel) and in cell lysates (bottom panels) with the monoclonal antibody pool M31,22,23 recognizing domains downstream of the breakpoint junction. Migration of the mature wild-type (matureWT-VWF) and deleted (matureDEL-VWF) proteins, and of forms containing the propeptide (proWT-VWF), is indicated. For comparison, see the Western blots in supplemental Figure 4. (B) VWF:Ag (white bars) and VWF:CB (gray bars) levels in conditioned media. The relative molar amount of pWT, pDEL, and vectors and the addition of siRNAs (40nM) are indicated in the table. 100% VWF:Ag corresponds to 445.1 ± 54.5 ng/mL and 100% of VWF:CB to 108.0% ± 14.7% of normal standard. Results from at least 3 independent experiments are reported as mean ± SEM. Statistical significance was evaluated by one-way analysis of variance with Bonferroni posttest: *P < .001; ○P < .01. nd indicates not detectable.

Wild-type and mutant VWF expression in cellular models. (A) Western blot analysis of wild-type and mutant VWF in conditioned media (top panel) and in cell lysates (bottom panels) with the monoclonal antibody pool M31,22,23  recognizing domains downstream of the breakpoint junction. Migration of the mature wild-type (matureWT-VWF) and deleted (matureDEL-VWF) proteins, and of forms containing the propeptide (proWT-VWF), is indicated. For comparison, see the Western blots in supplemental Figure 4. (B) VWF:Ag (white bars) and VWF:CB (gray bars) levels in conditioned media. The relative molar amount of pWT, pDEL, and vectors and the addition of siRNAs (40nM) are indicated in the table. 100% VWF:Ag corresponds to 445.1 ± 54.5 ng/mL and 100% of VWF:CB to 108.0% ± 14.7% of normal standard. Results from at least 3 independent experiments are reported as mean ± SEM. Statistical significance was evaluated by one-way analysis of variance with Bonferroni posttest: *P < .001; ○P < .01. nd indicates not detectable.

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