Nucleotide substitutions (AGT to GCC) within exon 19 result in the amino acid substitution of alanine for serine at position 743 (S743A). Flp-mediated recombination removed the loxP- and FRT-flanked neomycin resistance cassette inserted downstream of the modified exon 19. This results in a plasmin-inactivating active site mutation. Mice expression normal levels of protein in the plasma but no activity towards the Plm chromogenic substrate, S2251, despite formation of two-chain Plm. (J:210802)