This transgene contains a variant of human SOD1 that encodes a termination at codon position 103 (V103Z) with mutations at histidines at positions 46, 48, and 63 to R, Q, and G, respectively, to eliminate copper binding. In addition introns between exons 3, 4, and 5 of SOD1 were removed, creating a fragment with just two introns (one between exon 1 and 2 and one between exon 2 and 3) and exons 3, 4, and 5 fused to make one new exon. Two lines, E-17 and D-14, were established.