Shigatoxin triggers thrombotic thrombocytopenic purpura in genetically susceptible ADAMTS13-deficient mice
J. Clin. Invest. David G. Motto, et al. 115:2752 doi:10.1172/JCI26007 [
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Figure 1Generation of ADAMTS13-deficient mice. (
A) Schematic diagrams of the wild-type and targeted
Adamts13 alleles and the targeting vector. Exons are indicated as numbered boxes. The locations of PCR primers used in
B are indicated by lower-case letters and arrows. The locations of selected restriction sites are indicated by upper-case letters (E, EcoRI; H, HindIII; and S, SpeI). (
B) Genomic PCR demonstrating correct targeting of the
Adamts13 gene. Lanes 1, 4, and 7: primers a + b; lanes 2, 5, and 8: primers c + d + e; and lanes 3, 6, and 9: primers f + g. A 240-bp band specific for the
Neo insertion is seen only in
Adamts13+/– and
Adamts13–/– mice (lanes 4 and 7); a 280-bp band specific for the targeting vector is seen only in
Adamts13+/– and
Adamts13–/– mice (lanes 5 and 8); a 370-bp specific for the wild-type allele is seen only in
Adamts13+/+ and
Adamts13+/– mice (lanes 2 and 5); and a 400-bp band specific for deleted exon 6 is seen only in
Adamts13+/+ and
Adamts13+/– mice (lanes 3 and 6) and is not seen in
Adamts13–/– mice (lane 9). (
C) RT-PCR of liver mRNA prepared from
Adamts13B/129+/+,
Adamts13B/129+/–, and
Adamts13B/129–/– mice. Primers specific for exons 1–6 of
Adamts13 (upper panel) or vWF (lower panel) were used.
Adamts13 exons 1–6 were readily detected in mRNA from
Adamts13B/129+/+ and
Adamts13B/129+/– mice but were absent in mRNA from
Adamts13B/129–/– mice.
