Fus^tm2.1Neas

Exon 14 was flanked by loxP sites and part of exon 15 was replaced with a STOP cassette and human exon 15 sequence. The FRT-flanked neomycin resistance cassette that was inserted 50 bp downstream of the end of the 3 UTR was removed via flp-mediated recombination. This allele produces wild-type mouse protein. However, cre-mediated recombination can be used to remove exon 14 and modify the allele to reproduce an amyotrophic lateral sclerosis-associated human mutation (p.G466VfsX14) which leads to a reading frameshift and incorporation of 14 miscoded amino acids from exon 15 before an early termination codon and a C-terminal truncation. (J:326930)