Cystic Fibrosis Mice

Common CF mouse models include CFTR knockout and point mutation knockins modeling specific CFTR mutations found in patients (e.g., F508del, G551D). Models can be combined with conditional approaches for tissue specific CFTR expression or deletion to study organ specific disease mechanisms.

CF mice show intestinal obstruction (meconium ileus) and some lung pathology but do not fully recapitulate human lung disease severity. Models enable study of CFTR function, ion transport, and organ specific mechanisms, providing insights that complement human studies and enable preclinical testing.

Yes. We can generate point mutation knockin models carrying specific CFTR mutations found in patients (e.g., F508del, G551D, R117H). These models enable study of mutation-specific effects on CFTR folding, trafficking, and function, supporting development of mutation-specific therapeutics.