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TPP1 tripeptidyl peptidase I [Homo sapiens (human)]


RefSeq Accession Definition Service Stock Status Price *Turnaround time Order
NM_000391 Homo sapiens tripeptidyl peptidase I (TPP1), mRNA. GenEZ ORF Cloning On-demand $699.00 18

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Gene Symbol TPP1
Entrez Gene ID 1200
Full Name tripeptidyl peptidase I
Synonyms CLN2, LPIC, SCAR7, TPP-1
General protein information
Preferred Names
tripeptidyl-peptidase 1
Names
tripeptidyl-peptidase 1
tripeptidyl aminopeptidase
growth-inhibiting protein 1
cell growth-inhibiting gene 1 protein
lysosomal pepstatin insensitive protease
NP_000382.3
Gene Type protein-coding
Organism Homo sapiens (human)
Genome

11

11p15

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, Jul 2008].
Disorder

MIM: 607998

Ceroid-lipofuscinosis, neuronal 2, classic late infantile, 204500

mRNA Protein Product Sequence Price Select
NM_000391, 118582287 NP_000382, 5729770 tripeptidyl-peptidase 1 preproprotein ORF Sequence $550.00
KEGG
hsa04142Lysosome
Reactome
REACT_18356Unfolded Protein Response (UPR)
REACT_18368IRE1alpha activates chaperones
REACT_17015Metabolism of proteins
REACT_18273XBP1(S) activates chaperone genes
Homo sapiens (human)TPP1NP_000382.3
Pan troglodytes (chimpanzee)TPP1NP_001013025.1
Macaca mulatta (Rhesus monkey)LOC709838XP_001108748.1
Canis lupus familiaris (dog)TPP1NP_001013869.1
Bos taurus (cattle)TPP1NP_001069186.1
Mus musculus (house mouse)Tpp1NP_034036.1
Rattus norvegicus (Norway rat)Tpp1NP_112647.1
Gallus gallus (chicken)TPP1XP_423326.3
Danio rerio (zebrafish)tpp1NP_001122270.1
Xenopus (Silurana) tropicalis (western clawed frog)tpp1NP_001096294.1
Process
IDNameEvidence
GO:0006508proteolysisIMP
GO:0006629lipid metabolic processTAS
GO:0006987activation of signaling protein activity involved in unfolded protein responseTAS
GO:0007040lysosome organizationISS
GO:0007399nervous system developmentIMP
GO:0008219cell deathIEA
GO:0030163protein catabolic processNAS
GO:0030855epithelial cell differentiationIEP
GO:0030968endoplasmic reticulum unfolded protein responseTAS
GO:0043171peptide catabolic processIMP
GO:0044267cellular protein metabolic processTAS
GO:0045453bone resorptionIMP
GO:0050885neuromuscular process controlling balanceISS
Component
IDNameEvidence
GO:0005739mitochondrionIEA
GO:0005764lysosomeIDA
GO:0005764lysosomeIMP
GO:0042470melanosomeIEA
GO:0043202lysosomal lumenTAS
GO:0070062extracellular vesicular exosomeIDA
Function
IDNameEvidence
GO:0004175endopeptidase activityIDA
GO:0004175endopeptidase activityIMP
GO:0004252serine-type endopeptidase activityIEA
GO:0005515protein bindingIPI
GO:0008233peptidase activityIMP
GO:0008236serine-type peptidase activityIMP
GO:0008240tripeptidyl-peptidase activityIDA
GO:0008240tripeptidyl-peptidase activityIMP
GO:0042277peptide bindingISS
GO:0046872metal ion bindingIEA
GeneCards TPP1
UniProt O14773
Vega OTTHUMG00000133404
MIM 607998
Ensembl ENSG00000166340
HGNC 2073
HPRD 06415

GeneRIFs: Gene References Into Functions What's a GeneRIF?

What is the normal function of the TPP1 gene?

The TPP1 gene provides instructions for making an enzyme called tripeptidyl peptidase 1. This enzyme is produced as an inactive enzyme, called a proenzyme, which has an extra segment attached. This segment must be removed, followed by additional processing steps, for the enzyme to become active. The active tripeptidyl peptidase 1 enzyme is found in cell structures called lysosomes, which digest and recycle different types of molecules. This enzyme acts as a peptidase, which means that it breaks down protein fragments, known as peptides, into their individual building blocks (amino acids). Specifically, tripeptidyl peptidase 1 cuts (cleaves) peptides into groups of three amino acids.

Interactant

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