Tripeptidyl peptidase I

TPP1
Available structures
PDB	Ortholog search: PDBe RCSB
List of PDB id codes
	3EDY, 3EE6
Identifiers
Aliases	TPP1, CLN2, LPIC, SCAR7, TPP-1, GIG1, Tripeptidyl peptidase I, tripeptidyl peptidase 1
External IDs	OMIM: 607998; MGI: 1336194; HomoloGene: 335; GeneCards: TPP1; OMA:TPP1 - orthologs
Gene location (Human)
Chr.	Chromosome 11 (human)
End	6,619,448 bp
Gene location (Mouse)
Chr.	Chromosome 7 (mouse)
End	105,401,442 bp
RNA expression pattern
	Top expressed in
	retinal pigment epithelium; ; dorsal motor nucleus of vagus nerve; ; inferior olivary nucleus; ; right adrenal cortex; ; germinal epithelium; ; visceral pleura; ; stromal cell of endometrium; ; left adrenal gland; ; spleen; ; parietal pleura;
	Top expressed in
	choroid plexus of fourth ventricle; ; calvaria; ; stroma of bone marrow; ; tibiofemoral joint; ; retinal pigment epithelium; ; transitional epithelium of urinary bladder; ; decidua; ; right kidney; ; ciliary body; ; right lung;
	More reference expression data
	; ; ; ;
	More reference expression data
Gene ontology
Molecular function	tripeptidyl-peptidase activity; peptide binding; endopeptidase activity; metal ion binding; peptidase activity; protein binding; serine-type peptidase activity; serine-type endopeptidase activity; hydrolase activity;
Cellular component	melanosome; lysosomal lumen; mitochondrion; lysosome; extracellular exosome;
Biological process	epithelial cell differentiation; neuromuscular process controlling balance; lipid metabolism; bone resorption; nervous system development; proteolysis; central nervous system development; IRE1-mediated unfolded protein response; protein catabolic process; lysosome organization; peptide catabolic process;
	Sources:Amigo / QuickGO
Orthologs
	1200
	12751
	ENSG00000166340
	ENSMUSG00000030894
	O14773
	O89023
	NM_000391
	NM_009906
	NP_000382
	NP_034036
	Wikidata
View/Edit Human	View/Edit Mouse

Tripeptidyl-peptidase 1, also known as Lysosomal pepstatin-insensitive protease, is an enzyme that in humans is encoded by the TPP1 gene, also known as CLN2. TPP1 should not be confused with the TPP1 shelterin protein which protects telomeres and is encoded by the ACD gene. Mutations in the TPP1 gene leads to late-infantile neuronal ceroid lipofuscinosis.