• RHBDF2 (rhomboid 5 homolog 2), also known as RHBDL6 or iRhom2, belongs to a family of seven transmembrane-spanning proteins called rhomboids. The encoded protein regulates ADAM17 protease, a sheddase of the epidermal growth factor (EGF) receptor ligands and TNF, thereby plays a role in sleep, cell survival, proliferation, migration and inflammation.
• The exons 3-19 of mouse Rhbdf2 gene that encode the whole molecule were replaced by human counterparts in B-hRHBDF2 mice. The promoter, 5’UTR and 3’UTR region of the mouse gene are retained. The human RHBDF2 expression is driven by endogenous mouse Rhbdf2 promoter, while mouse Rhbdf2 gene transcription and translation will be disrupted.
• Mouse Rhbdf2 mRNA was detectable in wild-type C57BL/6JNifdc mice. Human RHBDF2 mRNA was detectable in homozygous B-hRHBDF2 mice.
• Human RHBDF2 was detectable in spleen, thymus, lung and bone marrow of homozygous humanized B-hRHBDF2 mice.
• Application: This product is used for preclinical pharmacological evaluation of antibody drugs.

Gene targeting strategy for B-hRHBDF2 mice. The exons 3-19 of mouse Rhbdf2 gene that encode the whole molecule were replaced by human counterparts in B-hRHBDF2 mice. The promoter, 5’UTR and 3’UTR region of the mouse gene are retained. The human RHBDF2 expression is driven by endogenous mouse Rhbdf2 promoter, while mouse Rhbdf2 gene transcription and translation will be disrupted.

Strain specific analysis of RHBDF2 mRNA expression in wild-type C57BL/6JNifdc mice and B-hRHBDF2 mice by RT-PCR. Lung RNA was isolated from wild-type C57BL/6JNifdc mice (+/+) and homozygous B-hRHBDF2 mice (H/H), then cDNA libraries were synthesized by reverse transcription, followed by PCR with mouse or human RHBDF2 primers. Mouse Rhbdf2 mRNA was only detectable in wild-type mice. Human RHBDF2 mRNA was exclusively detectable in homozygous B-hRHBDF2 mice but not in wild-type mice.

Strain specific RHBDF2 expression analysis in homozygous humanized B-hRHBDF2 mice by western blot. Spleen, lung, thymus, and bone marrow were collected from wild-type C57BL/6JNifdc mice (+/+) and homozygous B-hRHBDF2 mice (H/H) and analyzed by western blot with anti-RHBDF2 antibody which showed cross-reactivity between human and mouse (Proteintech, 23181-1-AP). Human RHBDF2 was detectable in spleen, lung, thymus, and bone marrow of wild-type C57BL/6JNifdc mice and homozygous B-hRHBDF2 mice.