The transgene contains the complete genomic sequence of the human superoxide dismutase 1, with a mutation in exon 4 changing codon 90 from aspartate to alanine (p.D90A). This mutation is the most common mutation found in amyotrophic lateral sclerosis (ALS) patients. Founders 134 and 154 had the highest transgene copy number and were selected for further study. The pound (#) sign is used when the specific line is not specified. (J:269950)