Tpp1 - tripeptidyl peptidase I Gene

Also known as Cln2; LPIC; TPP-1; TPP-I

About Tpp1

Summary

This gene encodes a lysosomal serine Protease that cleaves N-terminal Tripeptides from protein substrates. The encoded preproprotein undergoes autocatalytic processing to generate a mature Enzyme. Mice lacking the encoded protein exhibit a progressive neurodegeneration and a greatly shortened lifespan. At the cellular level, mice lacking the encoded protein exhibit accumulation of autofluorescent lipopigments. Mutations in the human ortholog of this gene cause classical late-infantile neuronal ceroid lipofuscinosis. [provided by RefSeq, Nov 2015]