These mice express a human aminopeptidase puromycin sensitive (hPSA or NPEPPS) gene directed by its endogenous promoter/enhancer regions on a BAC transgene. Mice hemizygous for the transgene are viable, fertile, normal in size and do not display any gross physical or behavioral abnormalities. PSA is a cytosolic aminopeptidase that hydrolyzes N-terminal amino acids. PSA is considered to have neuroprotective qualities due to its ability to degrade certain proteins associated with neurodegenerative diseases (TAU and polyglutamine expansion repeat-containing proteins). hPSA overexpression is seen in the cerebral cortex, spinal cord, cerebellum, hippocampus, and brain stem at a 2-3 fold greater level than endogenous PSA. hPSA activity is also increased in muscle, kidney, and liver. These mice may be useful for studying PSA protection in neurodegenerative disorders.

These mice express a human aminopeptidase puromycin sensitive (hPSA or NPEPPS) gene directed by its endogenous promoter/enhancer regions on the BAC transgene. These mice may be useful for studying PSA protection in neurodegenerative disorders.

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