tripeptidyl peptidase I

Symbol

TPP1 (may also be known as: None)

Organism

Human

Description

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]

Orthologs

SymbolSpecies
tpp1 Danio rerio
Tpp1 Mus musculus
Tpp1 Rattus norvegicus

Links to external resources

Changes associated with this gene

Identifier Name Type Tissues Organism Gene Data Actions
DAA3056 Gene expression of TPP1 decreases with age Molecular skin Human TPP1 —

GO Terms

GO IDGO TermGO Category
GO:0006508 proteolysis biological_process
GO:0006629 lipid metabolic process biological_process
GO:0006987 activation of signaling protein activity involved in unfolded protein response biological_process
GO:0007040 lysosome organization biological_process
GO:0007399 nervous system development biological_process
GO:0008219 cell death biological_process
GO:0030163 protein catabolic process biological_process
GO:0030968 endoplasmic reticulum unfolded protein response biological_process
GO:0043171 peptide catabolic process biological_process
GO:0045453 bone resorption biological_process
GO:0050885 neuromuscular process controlling balance biological_process
GO:0005625 soluble fraction cellular_component
GO:0005739 mitochondrion cellular_component
GO:0005764 lysosome cellular_component
GO:0042470 melanosome cellular_component
GO:0043202 lysosomal lumen cellular_component
GO:0004175 endopeptidase activity molecular_function
GO:0004252 serine-type endopeptidase activity molecular_function
GO:0005515 protein binding molecular_function
GO:0008233 peptidase activity molecular_function
GO:0008236 serine-type peptidase activity molecular_function
GO:0008240 tripeptidyl-peptidase activity molecular_function
GO:0042277 peptide binding molecular_function
GO:0046872 metal ion binding molecular_function