Tg(Prnp-Immt/SOD1)1Gmnf

The mito-WTSOD1 fusion protein contains a mouse mitofilin (Immt) cDNA sequence encoding the first 187 amino acids (including the mitochondrial targeting presequence, MMP cleavage site and transmembrane domain) fused in-frame to the N-terminus of a wild-type human Cu,Zn superoxide dismutase (SOD1) cDNA sequence. The SOD1 start codon was removed and substituted with an in-frame 6 nt DNA linker. This mito-WTSOD1 sequence was inserted between exon 2 and exon 3 of mouse prion protein (PrP or Prnp) gene at a unique XhoI site in the MoPrP.Xho plasmid vector. The founder line with the highest expression level (founder line 1) was characterized. (J:211695)