Tg(SOD1*L126Z)45Dbo

A 12 kb genomic fragment of human SOD1 with introns between exons 3, 4, and 5 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, and a leucine to glutamine/glutamic acid substitution at position 126 (L126Z) in exon 5. This mutation results in a premature stop codon at position 126 and produces a truncated product that lacks the C-terminal 28 amino acids. Three lines were made: 171, 45, and 44. (J:219482)