Gene targeting strategy for B-hGEP mice. The exons 2-13 of mouse Gep gene that encode the full-length protein were replaced by human GEP exons 2-13 in B-hGEP mice.

Strain specific GEP expression analysis in homozygous B-hGEP mice by ELISA. Serum were collected from wild-type mice C57BL/6 mice (+/+) and homozygous B-hGEP mice (H/H), and analyzed by ELISA with species-specific Progranulin ELISA kit. Mouse Progranulin was detectable in wild-type mice (A). Human Progranulin was exclusively detectable in homozygous B-hGEP mice but not in wild-type mice (B).