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TPP1 tripeptidyl peptidase I [Homo sapiens (human)]


Clone ID RefSeq Accession Definition Vector Stock Status *Turnaround time Price Order
OHu28414 NM_000391 Homo sapiens tripeptidyl peptidase I (TPP1), mRNA. pcDNA3.1+-DYK On-demand 7-9 $219.00
OHu28414C NM_000391 Homo sapiens tripeptidyl peptidase I (TPP1), mRNA. Customized vector On-demand 7-9 $269.00

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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?

Interactant

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