Exon 2 was replaced with a modified exon 2 in which nucleotide substitutions (CTT to AAT at codon 7 and CTC to AAC at codon 17) result in the amino acid substitutions of asparagine for leucine at positions 7 and 17 (L7N and L17N) in the SRP-binding and ER transmembrane anchor/stop transfer sequence. A floxed neo cassette inserted upstream of the modified exon was removed by cre mediated recombination. The product is predicted to undergo mitochondrial proteolysis targeting. (J:163973)