The transgene contains the entire genomic sequence of human SOD1 with a 2-bp deletion at codon 126 and a FLAG sequence at the C-terminal. The mutation causes a frame shift and results in premature stop sequence at codon 131, resulting in a mutant protein of 130 amino acids. No SOD1 activity could be detected, however, Western and Northern analysis indicated protein expression and presence of RNA, respectively. Two lines were established, DF2 and DF7. (J:97932)