Gene targeting strategy for B-hGPC1 mice. The exons 2-9 of mouse Gpc1 gene that encode the full-length protein without signal peptide were replaced by human GPC1 exons 2-9 in B-hGPC1 mice.

Strain specific analysis of GPC1 gene expression in wild-type mice and B-hGPC1 mice by RT-PCR. Mouse Gpc1 mRNA was detectable only in lung of wild-type mice (+/+). Human GPC1 mRNA was detectable only in homozygous B-hGPC1 mice (H/H) but not in wild-type mice (+/+).

Strain specific GPC1 expression analysis in homozygous B-hGPC1 mice by western blot. Heart was collected from wild-type mice (+/+) and homozygous B-hGPC1 mice (H/H), and analyzed by western blot with anti-GPC1 antibody. Human GPC1 was detectable in homozygous B-hGPC1 mice (H/H).