TPP2

This gene encodes a mammalian peptidase that, at neutral pH, removes tripeptides from the N terminus of longer peptides. The protein has a specialized function that is essential for some MHC class I antigen presentation. The protein is a high molecular mass serine exopeptidase; the amino acid sequence surrounding the serine residue at the active site is similar to the peptidases of the subtilisin class rather than the trypsin class. [provided by RefSeq, Jul 2008]

Full Name

TPP2 Tripeptidyl Peptidase 2

Function

Cytosolic tripeptidyl-peptidase that releases N-terminal tripeptides from polypeptides and is a component of the proteolytic cascade acting downstream of the 26S proteasome in the ubiquitin-proteasome pathway (PubMed:25525876, PubMed:30533531).
It plays an important role in intracellular amino acid homeostasis (PubMed:25525876).
Stimulates adipogenesis (By similarity).

Biological Process

Biological Process amino acid homeostasis Source:UniProtKB1 Publication
Biological Process protein polyubiquitination Source:Reactome
Biological Process proteolysis Source:ProtInc1 Publication

Cellular Location

Cytoplasm
Nucleus
Translocates to the nucleus in response to gamma-irradiation.

Involvement in disease

Immunodeficiency 78 with autoimmunity and developmental delay (IMD78):
An autosomal recessive disorder characterized by immune dysregulation, increased susceptibility to bacterial, viral and fungal infections, recurrent sinopulmonary or skin infections, and autoimmune abnormalities including hemolytic anemia and autoimmune cytopenias. Patients also have global developmental delay with speech delay and variable intellectual disability. Disease onset is in infancy or early childhood.