InnateDB: Systems Biology of the Innate Immune Response

Homo sapiens Gene: TPP1

Summary

InnateDB Gene

IDBG-29461.6

Last Modified

2014-10-13 [Report errors or provide feedback]

Gene Symbol

TPP1

Gene Name

tripeptidyl peptidase I

Synonyms

CLN2; LPIC; SCAR7; TPP-1

Species

Homo sapiens

Ensembl Gene

ENSG00000166340

Encoded Proteins

IDBP-29463

tripeptidyl peptidase I

IDBP-365080

tripeptidyl peptidase I

IDBP-590574

tripeptidyl peptidase I

IDBP-599962

tripeptidyl peptidase I

IDBP-587710

tripeptidyl peptidase I

IDBP-813037

tripeptidyl peptidase I

Protein Structure

Useful resources

Stemformatics EHFPI ImmGen

Entrez 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, Jul 2008]

Gene Information

Type

Protein coding

Genomic Location

Chromosome 11:6612763-6619461

Strand

Reverse strand

Band

p15.4

Transcripts

ENST00000299427	ENSP00000299427
ENST00000436873	ENSP00000398136
ENST00000428886
ENST00000533371	ENSP00000437066
ENST00000524611
ENST00000532191
ENST00000524924
ENST00000528807
ENST00000524788
ENST00000528571	ENSP00000434647
ENST00000534644
ENST00000530040
ENST00000528917
ENST00000531754
ENST00000524903
ENST00000528657	ENSP00000435001
ENST00000611494	ENSP00000484546

Interactions

Number of Interactions

This gene and/or its encoded proteins are associated with 22 experimentally validated interaction(s) in this database.
They are also associated with 1 interaction(s) predicted by orthology.

Experimentally validated
Total	22 [view]
Protein-Protein	21 [view]
Protein-DNA	1 [view]
Protein-RNA	0
DNA-DNA	0
RNA-RNA	0
DNA-RNA	0

Predicted by orthology
Total	1 [view]

Gene Ontology

Molecular Function

Accession	GO Term
GO:0004175	endopeptidase activity
GO:0004252	serine-type endopeptidase activity
GO:0005515	protein binding
GO:0008233	peptidase activity
GO:0008236	serine-type peptidase activity
GO:0008240	tripeptidyl-peptidase activity
GO:0042277	peptide binding
GO:0046872	metal ion binding

Biological Process

GO:0006508	proteolysis
GO:0006629	lipid metabolic process
GO:0006987	activation of signaling protein activity involved in unfolded protein response
GO:0007040	lysosome organization
GO:0007399	nervous system development
GO:0008219	cell death
GO:0030163	protein catabolic process
GO:0030855	epithelial cell differentiation
GO:0030968	endoplasmic reticulum unfolded protein response
GO:0043171	peptide catabolic process
GO:0044267	cellular protein metabolic process
GO:0045453	bone resorption
GO:0050885	neuromuscular process controlling balance

Cellular Component

GO:0005739	mitochondrion
GO:0005764	lysosome
GO:0042470	melanosome
GO:0043202	lysosomal lumen
GO:0070062	extracellular vesicular exosome

Orthologs

Species

Mus musculus

Bos taurus

Gene ID

Gene Order

ENSMUSG00000030894

View Gene Order

ENSBTAG00000015403

Not yet available

Pathways

NETPATH

REACTOME

REACT_18273

XBP1(S) activates chaperone genes pathway

REACT_18368

IRE1alpha activates chaperones pathway

REACT_18356

Unfolded Protein Response (UPR) pathway

REACT_17015

Metabolism of proteins pathway

KEGG

hsa04142

Lysosome pathway

INOH

PID NCI

Cross-References

SwissProt

TrEMBL

E9PME9

UniProt Splice Variant

Entrez Gene

1200

UniGene

RefSeq

NM_000391

HUGO

HGNC:2073

OMIM

607998

CCDS

CCDS7770

HPRD

IMGT

EMBL

AC091564

GenPept

RNA Seq Atlas

1200