The PrP-mitoSOD1^WT transgene (PrP-mito-WTSOD1 or mitoSOD1) was designed by Drs. Giovanni Manfredi and Jordi Magrane (Weill Medical College of Cornell University). 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 wildtype 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. In contrast to the publication, the donating investigator reports the resulting PrP-mito-WTSOD1 transgene was microinjected into [C57BL/6J x CBA/J]F2 fertilized eggs. Male transgenic founders were bred with B6SJLF1/J female mice, and and the founder line with the highest expression level (founder line 1) was characterized. At that time, quantitative real-time PCR determined transgenic animals had ~45 transgene copies, and breeding performance indicated autosomal integration of the transgenes. The mito-WTSOD1 transgenic colony was maintained by breeding hemizygous males with B6SJLF1/J females for at least ten generations prior to sending males to The Jackson Laboratory Repository in 2014. Upon arrival, sperm was cryopreserved. To generate our live colony, an aliquot of the frozen sperm was used to fertilize oocytes from B6SJLF1/J mice (Stock No. 100012). Thereafter, the colony was maintained by breeding hemizygous mice with B6SJLF1/J mice every generation.

• Noncarrier

Approximate Controls

• 100012 B6SJLF1/J

Additional Information

• Considerations for Choosing Controls

• Magrane J; Cortez C; Gan WB; Manfredi G. 2014. Abnormal mitochondrial transport and morphology are common pathological denominators in SOD1 and TDP43 ALS mouse models. Hum Mol Genet 23(6):1413-24PubMed: 24154542MGI: J:206221