Slc25a4^tm1.1Xjc

A loxP site was inserted into intron 1, an FRT site flanked neomycin resistance gene cassette, a second loxP site and a mini-gene (containing the last 254 bp of intron 1 sequence (including the splice acceptor), exon 2 with p.A114P and p.A123D mutations, exons 3 and 4, and 70 bp of downstream sequence) were inserted downstream of the gene. The neo cassette was removed through subsequent Flp-mediated recombination. This allele only expresses the mini-gene encoding the mutant peptide after Cre-mediated deletion of endogenous exons 2-4. The mutations interfere with the ability of the encoded peptide to cross mitochondrial membranes. (J:338762)