The neurodevelopmental disorder Williams-Beuren Syndrome (WBS) is caused by spontaneous 1.5 Mb deletions comprising 25 genes on human chromosome 7q11.23. This mutant mouse strain carries a deletion corresponding to the distal half of the conserved syntenic region on mouse Chromosome 5G2, referred to as the distal deletion (DD).
Karl Deisseroth, Stanford University
The neurodevelopmental disorder Williams-Beuren Syndrome (WBS) is caused by spontaneous1.5 Mb deletions comprising 25 genes on human chromosome 7q11.23. This mutant mouse strain carries a deletion corresponding to the proximal half of the conserved syntenic region on mouse Chromosome 5G2, referred to as the distal deletion (DD).
DD mice lack a genomic segment between and including the Limk1 to Trim50 genes. Crosses with animals lacking genomic sequences corresponding to the proximal portion of the WBS deletion region (see Stock No. 023885; also called PD) create double heterozygous animals with deletions representing the length of the WBS region (D/P mice). Both PD and D/P males are growth-retarded, while skulls are shortened and brains are smaller in DD and D/P. Lateral ventricle volumes are reduced, and neuronal cell density in the somatosensory cortex is increased, in PD and D/P. Motor skills are most impaired in D/P animals and their fertility is somewhat reduced. Together, these partial deletion mice replicate crucial aspects of the human disorder and serve to identify genes and gene networks contributing to the neural substrates of complex behaviours and behavioural disorders.
A genomic segment corresponding to the distal portion of the Williams-Beuren Syndrome (WBS) deletion region was excised in these mice. To create the mutation, a neomycin resistance cassette, a loxP site, the 5' portion of the Hprt minigene, and Limk1 exons 3 and 4 were introduced to intron 2 of the Limk1 locus. The 3' portion of the Hprt minigene, loxP site and puromycin resistance cassette were inserted into intron 2 of the Trim50 gene. These mutations were introduced through homologous recombination to AB2.2 129S7/SvEvBrd-Hprt1b-m2-derived embryonic stem (ES) cells. Resultant chimeric mice were crossed with Zp3-cre transgenic mice (see Stock No. 003651) to excise the genomic region flanked by loxP sites. These mice were maintained on a mixed C57BL/6J and 129 genetic background by the donating laboratory.
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