Sftpc^tm3.1Mfbs

Cysteine codon 185 (TGT) in exon 5 was changed to glycine (GGT) (p.C185G) and a loxP site flanked neomycin resistance gene was inserted into intron 4. The neo cassette was removed through subsequent Cre-mediated recombination. The mutation, in the BRICHOS domain of the encoded peptide, prevents the formation of the disulfide bridge with C121 that is essential for correct folding of the pro-protein and associates with respiratory failure in neonate, interstitial lung disease in children, and fibrotic remodeling in adults. (J:335487)