Mmut^tm1.1Mrb

A targeting vector was designed to replace thymine with adenine at position 2093, resulting in a methionine to lysine mutation at amino acid 698 (M698K) in exon 12, which corresponds to the human M700L missense mutation seen in some methylmalonic aciduria patients. A FRT-flanked neomycin selection cassette was inserted downstream of exon 12 and was removed via FLP-mediated recombination. Western blot analysis indicates significantly reduced level of protein in homozygotes and Mut enzyme activity is greatly decreased. (J:237040)