Entrez 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].
UniProt Summary: Lysosomal serine protease with tripeptidyl-peptidase I activity. May act as a non-specific lysosomal peptidase which generates tripeptides from the breakdown products produced by lysosomal proteinases. Requires substrates with an unsubstituted N-terminus (By similarity). {ECO:0000250|UniProtKB:Q9EQV6}.
Pfam DomainsGO Terms
Pfam Domains
Peptidase S8
Pro-kuma activ
GO Terms
tripeptidyl-peptidase activity
bone resorption
peptide catabolic process
bone remodeling
peptide binding
neuromuscular process controlling balance
IRE1-mediated unfolded protein response
lytic vacuole organization
serine-type peptidase activity
lysosome organization
endopeptidase activity
peptidase activity
lysosomal lumen
tissue remodeling
melanosome
endoplasmic reticulum unfolded protein response
neuromuscular process
cellular response to unfolded protein
vacuole organization
cellular response to topologically incorrect protein
response to unfolded protein
serine-type endopeptidase activity
response to topologically incorrect protein
tissue homeostasis
lysosome
response to endoplasmic reticulum stress
multicellular organismal homeostasis
anatomical structure homeostasis
peptide metabolic process
epithelial cell differentiation
protein catabolic process
cellular amide metabolic process
central nervous system development
macromolecule catabolic process
organonitrogen compound catabolic process
epithelium development
lipid metabolic process
proteolysis
nervous system process
homeostatic process
cellular response to stress
tissue development
organic substance catabolic process
cellular catabolic process
system process
CRISPR Data
Compound HitMost Correlated Genes in ChemogenomicsTissues where Essential in the Avana Dataset (DepMap 20Q1)
Compound Hit
No hits were found.
Most Correlated Genes in Chemogenomics
No correlation found to any other genes in chemogenomics.
Tissues where Essential in the Avana Dataset (DepMap 20Q1)
Global Fraction of Cell Lines Where Essential: 0/739