Mice homozygous for this targeted mutation are viable and fertile. On the C57BL/6 background, the mice have a mild procoagulant phenotype using the ferric chloride vascular injury model, and von Willebrand factor (VWF)-mediated platelet interactions are slightly prolonged compared to wildtype (using intravital microscopy). There is no sponataneous thrombotic thrombocytopenic purpura (TTP) phenotype. Expression of the targeted exons has been eliminated in the liver, as tested by RT-PCR. There is no detectable activity of the enzyme in the plasma of homozygous targeted mice.
A targeting vector was designed to replace exons 1-6 with a PGK/Neomycin cassette. The construct was electroporated into (129X1/SvJ x 129S1/Sv)F1- Kitl+-derived R1 embryonic stem (ES) cells. Targeted clones were injected into C57BL/6J blastocysts. The strain was backcrossed twelve times to C57BL/6 by the donating laboratory.
|Allele Name||targeted mutation 1, David Ginsburg|
|Allele Type||Targeted (Null/Knockout)|
|Gene Symbol and Name||Adamts13, a disintegrin-like and metallopeptidase (reprolysin type) with thrombospondin type 1 motif, 13|
|Strain of Origin||129X1/SvJ|
|Molecular Note||A neomycin resistance gene replaced exons 1-6. RT-PCR of mutant liver samples confirmed absence of transcript.|
|Mutations Made By|| |
David Ginsburg, University of Michigan
When maintained as a live colony, homozygotes may be bred.
When using the B6.129-Adamts13tm1Dgi/J mouse strain in a publication, please cite the originating article(s) and include JAX stock #007235 in your Materials and Methods section.