Products

Western Blot (Transfected lysate)

Immunofluorescence (Transfected cell)

Flow Cytometry (Transfected cell)

Entrez GeneID

GeneBank Accession#

Protein Accession#

Gene Name

TPP1

Gene Alias

CLN2, GIG1, LPIC, MGC21297

Gene Description

tripeptidyl peptidase I

Omim ID

Gene Ontology

Gene Summary

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

Other Designations

ceroid-lipofuscinosis, neuronal 2, late infantile (Jansky-Bielschowsky disease)|growth-inhibiting protein 1|lysosomal pepstatin insensitive protease|tripeptidyl aminopeptidase|tripeptidyl-peptidase I