Fus^tm1.1Neas

A proline to leucine substitution at amino acid 527 (p.P517L) was introduced in exon 15. 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 mutation is equivalent to the human P525L allele associated with a rapidly progressive, juvenile-onset form of amyotrophic lateral sclerosis. (J:326930)