Slc25a39fl/fl mice carry a CRISPR/Cas9 generated mutation resulting in loxP sites flanking exons 2-3, including the translation initiation codon, of the solute carrier family 25, member 39 (Slc25a39) gene. Slc25a39 encodes a mitochondrial membrane carrier required for glutathione (GSH) import into mitochondria and SLC25a39 protein levels are regulated by GSH availability. GSH is a component of antioxidant defense systems that plays roles in disulfide bond formation, detoxification of xenobiotics, metabolite transport, formation of iron-sulfur clusters and cellular signaling. GSH metabolism has been implicated in some diseases such as diabetes, cystic fibrosis, cancer, and some neurodegenerative diseases. Mice that are homozygous for this allele are viable and fertile. When bred to mice that express tissue-specific Cre recombinase, resulting offspring will have exons 2-3, including the translation initiation codon, removed in the Cre-expressing tissues of the offspring. When Slc25a39fl/fl mice are bred with ErGFP-cre mice, with cre expression in erythroid cells, the resulting Slc25a39-KO mice exhibit severe anemia, iron
overload and increased apoptosis in fetal liver cells. There is also a robust reduction in the number of erythroblasts at all differentiation stages, with no defects evident in other lineages.

Slc25a39fl mice carry a CRISPR/Cas9 generated mutation resulting in loxP sites flanking exons 2-3, including the translation initiation codon, of the Slc25a39 gene. This strain may be useful for studying the regulation of regulated mitochondrial glutathione import.