The tripeptidyl peptidase I (Tpp1) gene codes for a lysosomal peptidase that cleaves N-terminal tripeptides from proteins. Mutations of this gene results in a build-up of cellular waste that leads to a neurological disorder, classical late-infantile neuronal ceroid lipofuscinosis (cLINCL). cLINCL patients display epileptic seizures, ataxia, vision loss and show developmental regression. These TgLSL-Tpp1 mice carry a loxP-flanked stop cassette, followed by mouse Tpp1 cDNA, inserted into the Gt(ROSA)26Sor locus. Prior to Cre-mediated recombination, the TgLSL-Tpp1 mice express TPP1 at levels similar to wildtype controls. The donating laboratory has shown that after Cre-recombination with germline Zp3-cre mouse line (Stock No. <a href="https://www.jax.org/strain/003651">003651</a>), recombined heterozygous mice, referred to as TgL-Tpp1 overexpress TPP1 by 10- and 50-fold in brain and liver respectively, with no overt phenotype. Heterozygous TgLSL-Tpp1 mice are viable and fertile with no overt phenotype. Homozygous mice have not been evaluated by the donating laboratory (December 2023). These mice may be useful for studying Cre-inducible overexpression of Tpp1 as it relates to lysosomal storage diseases such as classical late-infantile neuronal ceroid lipofuscinosis (cLINCL).

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TgLSL-TPP1 knock-in mice harbor a floxed stop cassette, followed by mouse tripeptidyl peptidase I cDNA (Tpp1), knocked-in to the Gt(ROSA)26Sor locus. These mice may be useful for studying the role of the Tpp1 overexpression in cre-expressing tissues, specifically as it relates to the lysosomal storage disease classical late-infantile neuronal ceroid lipofuscinosis (cLINCL).

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