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. Author manuscript; available in PMC: 2011 Jan 1.
Published in final edited form as: Mol Genet Metab. 2010 Jan;99(1):62–71. doi: 10.1016/j.ymgme.2009.08.002

Figure 1.

Figure 1

Generation of the Idua-W392X knock-in mouse. (A) The Idua-W392X targeting construct (upper) was integrated into the mouse Idua locus (lower) by homologous recombination (dashed lines). The bold horizontal lines indicate the targeted region. The bold vertical lines indicate exons (not shown to scale). Exons 3, 9, and 14 are numbered. The W392X mutation was introduced into exon 9. The sizes of the genomic DNA fragments after EcoRI digestion in both wild-type and targeted Idua loci are indicated as well as the region of probe binding used in Southern blotting. tk: thymidine kinase gene; neo: neomycin resistance gene (flanked by loxP sites shown as gray bars). (B) Southern blot of wild-type and two targeted ES cell clones (denoted as F8 and H4) resulted in a 9.9 kb fragment from the wild-type Idua locus and a 4.2 kb fragment from the targeted Idua locus. (C) α-L-iduronidase specific activity determined in wild-type and two targeted ES cell clones. (D) PCR of the Idua allele using genomic DNA derived from tail snips (following Cre recombinase-mediated excision of the neo cassette) results in a 576 bp product from the wild-type Idua allele and a 610 bp product from the W392X allele.