hCFB Mouse Model
| Strain Name | hCFB Mouse Model |
|---|---|
| Strain Background | C57BL/6N |
Applications
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Complement pathway research: Study C3bBb convertase assembly and amplification.
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CFB drug evaluation: Test anti-CFB ASOs, siRNAs, and small-molecule inhibitors.
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Kidney disease modeling: Model C3 glomerulopathy and IgA nephropathy.
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PK/PD & dose finding: Link hepatic CFB knockdown to plasma levels and complement activity.
Strain Description
The hCFB Mouse Model carries a targeted knock-in of the human CFB gene at the endogenous mouse locus. It replaces the murine Cfb coding sequence while preserving native promoters and regulatory elements. Therefore, it ensures physiological expression of functional human complement factor B in the same cell types where the endogenous gene is normally expressed, primarily hepatocytes. This human CFB knock-in mouse produces human factor B that participates in alternative pathway activation in a physiologically relevant manner.
In this hCFB model, factor B binds C3b to form the C3 convertase (C3bBb) after cleavage by factor D. This enzyme amplifies C3b deposition and drives terminal complement activation via C5 convertase assembly. Furthermore, dysregulated CFB activity promotes excessive C3 fragment accumulation in glomerular capillaries—a hallmark of C3 glomerulopathy and IgA nephropathy. Loss of CFB in the hCfb mouse reduces alternative pathway activity and protects against complement-mediated renal injury.
This humanized CFB model allows therapeutics to engage authentic human CFB sequences. For example, you can test antisense oligonucleotides (ASOs) or GalNAc-siRNAs designed to silence human CFB in hepatocytes. Similarly, it supports evaluation of small-molecule CFB inhibitors such as iptacopan analogs. Consequently, the hCFB Mouse Model provides a translational platform for CFB-targeted drug efficacy, dose–response, and complement inhibition studies that conventional models cannot replicate.
FAQ
Game-changing benefits？
While competitors highlight germline efficiency gains, shorter timelines and enhanced 3Rs animal welfare benefits for their technologies, these are merely incremental improvements over traditional approaches. In sharp contrast, our proprietary technology delivers fully pure, homogeneous lineages—every single cell of the mice is derived exclusively from totipotent ES cells, with guaranteed 100% germline transmission efficiency. To experience these unparalleled benefits firsthand, enquire about your custom mouse model project with us or order embryos for in-house validation at your facility.
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