Tripeptidyl peptidase I

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Tripeptidyl peptidase I
Available structures
PDB Ortholog search: PDBe, RCSB
Symbols TPP1 ; CLN2; LPIC; SCAR7; TPP-1
External IDs OMIM607998 MGI1336194 HomoloGene335 GeneCards: TPP1 Gene
EC number
RNA expression pattern
PBB GE TPP1 200743 s at tn.png
PBB GE TPP1 200742 s at tn.png
PBB GE TPP1 214196 s at tn.png
More reference expression data
Species Human Mouse
Entrez 1200 12751
Ensembl ENSG00000166340 ENSMUSG00000030894
UniProt O14773 O89023
RefSeq (mRNA) NM_000391 NM_009906
RefSeq (protein) NP_000382 NP_034036
Location (UCSC) Chr 11:
6.63 – 6.64 Mb
Chr 7:
105.74 – 105.75 Mb
PubMed search [1] [2]

Tripeptidyl-peptidase 1 is an enzyme that in humans is encoded by the TPP1 gene.[1][2]


Molecular biology[edit]

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 autoproteolyzed upon acidification.[2]


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.


  1. ^ Liu CG, Sleat DE, Donnelly RJ, Lobel P (Oct 1998). "Structural organization and sequence of CLN2, the defective gene in classical late infantile neuronal ceroid lipofuscinosis". Genomics 50 (2): 206–12. doi:10.1006/geno.1998.5328. PMID 9653647. 
  2. ^ a b "Entrez Gene: TPP1 tripeptidyl peptidase I". 

External links[edit]

Further reading[edit]