Tg(SOD1*G85R/EYFP)641Alho

A 12 kb genomic fragment of human SOD1 carrying the glycine to arginine substitution at amino acid 85 (G85R) fused to EYFP coding sequence through its C-terminal coding sequence in exon 5 was used as the transgene. The G85R mutation results in a misfolded protein associated with amyotrophic lateral sclerosis in humans and the mutant fusion protein behaves as a misfolded monomer. Southern blot analysis indicates that line 641 carries a single (multicopy) transgene insertion. In situ RNA hybridization indicates that the transgene is predominantly in motor neurons in the spinal cord. (J:144475)