Figure 6.
Autoradiograph of recombinant VWF multimers. rVWF protein secreted into the media was concentrated and multimer analysis was performed. (A) Lane 1, 100% wild type; lane 2, heterozygous (cotransfection of wild-type and pCIneoG1180R plasmid); and lane 3, 100% G1180R mutant. The G1180R mutant protein does not result in a loss of HMW multimers. (B) Lane 1, 100% wild type; lane 2, heterozygous (cotransfection of wild-type and pCIneodel26 plasmid); lane 3, heterozygous (cotransfection of wild-type and pCIneodel23/26 plasmid); lane 4, homozygous pCIneodel26 mutant; lane 5, homozygous del23/26 mutant; and lane 6, 100% wild type. Both of the homozygous transfections show the presence of only the lowest molecular weight multimers, whereas the heterozygous transfections show a normal multimeric pattern.

Autoradiograph of recombinant VWF multimers. rVWF protein secreted into the media was concentrated and multimer analysis was performed. (A) Lane 1, 100% wild type; lane 2, heterozygous (cotransfection of wild-type and pCIneoG1180R plasmid); and lane 3, 100% G1180R mutant. The G1180R mutant protein does not result in a loss of HMW multimers. (B) Lane 1, 100% wild type; lane 2, heterozygous (cotransfection of wild-type and pCIneodel26 plasmid); lane 3, heterozygous (cotransfection of wild-type and pCIneodel23/26 plasmid); lane 4, homozygous pCIneodel26 mutant; lane 5, homozygous del23/26 mutant; and lane 6, 100% wild type. Both of the homozygous transfections show the presence of only the lowest molecular weight multimers, whereas the heterozygous transfections show a normal multimeric pattern.

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