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TPP1 tripeptidyl peptidase 1

Also known as: CLN2; GIG1; LPIC; SCAR7; TPP-1


This gene encodes a member of the sedolisin family of serine proteases. The protease functions in the lysosome to cleave N-terminal tripeptides from substrates, and has weaker endopeptidase activity. It is synthesized as a catalytically-inactive enzyme which is activated and auto-proteolyzed upon acidification. Mutations in this gene result in late-infantile neuronal ceroid lipofuscinosis, which is associated with the failure to degrade specific neuropeptides and a subunit of ATP synthase in the lysosome. [provided by RefSeq, Jul 2008]

Genomic context

Chromosome: 11; NC_000011.10 (6612766..6619461, complement)
Total number of exons:


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