• Heat Shock Protein 47 (HSP47), also known as Serpin-H1, is localized to endoplasmic reticulum (ER), where it is a collagen-specific molecular chaperone.
• In the ER, HSP47 interacts with and stabilizes correctly-folded procollagen, which is up-regulated in several cancers including squamous cell carcinoma, breast and prostate carcinomas.
• The exons 1-5 of mouse Serpinh1 gene that encode the whole molecule (ATG to STOP codon), including promoter, 5’UTR and 3’UTR were replaced by human counterparts in B-hSERPINH1 mice. The human SERPINH1 expression is driven by human Serpinh1 promoter, while mouse Serpinh1 gene transcription and translation will be disrupted.
• SERPINH1 protein was detectable in in heart, liver(weak expression), lung(weak expression), kidney(weak expression), uterus and ovary of wild-type C57BL/6 mice and homozygous B-hSERPINH1 mice, as the antibody can cross-recognize both human and mouse SERPINH1.
• Mouse Serpinh1 mRNA was detectable in wild-type C57BL/6 mice. Human SERPINH1 mRNA was detectable in homozygous B-hSERPINH1 mice.

Gene targeting strategy for B-hSERPINH1 mice. The exons 1-5 of mouse Serpinh1 gene that encode the whole molecule (ATG to STOP codon), including promoter, 5’UTR and 3’UTR were replaced by human counterparts in B-hSERPINH1 mice. The human SERPINH1 expression is driven by human SERPINH1 promoter, while mouse Serpinh1 gene transcription and translation will be disrupted.

Species specific analysis of SERPINH1 gene expression in wild-type C57BL/6 mice and homozygous humanized B-hSERPINH1 mice by RT-PCR. Lung was collected from wild-type C57BL/6 mice (+/+) and homozygous B-hSERPINH1 mice (H/H). Mouse Serpinh1 mRNA was detectable in wild-type C57BL/6 mice. Human SERPINH1 mRNA was detectable only in homozygous B-hSERPINH1 mice, but not in wild-type C57BL/6 mice.

Strain specific SERPINH1 expression analysis in homozygous B-hSERPNH1 mice by western blot. Heart, liver, lung, kidney, uterus and ovary were collected from wild-type C57BL/6 mice (+/+) and homozygous B-hSERPINH1 mice (H/H), and analyzed by western blot with anti-SERPINH1 antibody (proteintech, 10875-1-AP). m/hSERPINH1 was detectable in heart, liver(weak expression), lung(weak expression), kidney(weak expression), uterus and ovary of wild-type C57BL/6 mice and homozygous B-hSERPINH1 mice.