Slc13a5 (solute carrier family 13 (sodium-dependent citrate transporter), member 5) is a sodium-dependent citrate cotransporter important in maintaining various metabolic processes. Mutations in SLC13A5 are associated with a citrate transport disorder, a rare neurological disease, and a rare epilepsy, developmental and epileptic encephalopathy 25 with amelogenesis imperfecta. Slc13a5em1(SLC13A5)Lutzy knockin/knockout mice express a complete humanized WT cDNA with bGH poly(A) transcription termination signal replacing the endogenous coding exon 1 of the Slc13a5 gene. The insertion prevents translation of the mouse Slc13a5 cDNA. Homozygous mice are viable and fertile.

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Slc13a5em1(SLC13A5)Lutzy knockin/knockout mice express a complete wild-type human SLC13A5 cDNA. The insertion disrupts expression of endogenous Slc13a5. These mice may be useful for studying neurological diseases associated with citrate transport.

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