Recombinant Human Probable Xaa-Pro aminopeptidase 3 (XPNPEP3)

Uniprot Id

Q9NQH7

Target Species

Human

Target Name

XPNPEP3

Target Full Name

Xaa-Pro aminopeptidase 3

Target Function

Catalyzes the removal of a penultimate prolyl residue from the N-termini of peptides, such as Leu-Pro-Ala. Also shows low activity towards peptides with Ala or Ser at the P1 position.; Promotes TNFRSF1B-mediated phosphorylation of MAPK8/JNK1 and MAPK9/JNK2, suggesting a function as an adapter protein for TNFRSF1B; the effect is independent of XPNPEP3 peptidase activity. May inhibit apoptotic cell death induced via TNF-TNFRSF1B signaling.

Target Involvement

Nephronophthisis-like nephropathy 1 (NPHPL1)

Target Subcellular Location

[Isoform 1]: Mitochondrion. Cytoplasm.; [Isoform 2]: Cytoplasm.

Target Protein Families

Peptidase M24B family

Target Tissue Specificity

Isoform 1 and isoform 2 are widely expressed, with isoform 1 being more abundant.

Target Synonyms

Aminopeptidase P3; APP3; NPHPL1; OTTHUMP00000199806; Probable Xaa Pro aminopeptidase 3; Probable Xaa-Pro aminopeptidase 3; X Pro aminopeptidase 3; X prolyl aminopeptidase (aminopeptidase P) 3; X-Pro aminopeptidase 3; XPNPEP3; XPP3_HUMAN

Target Background

The protein encoded by this gene belongs to the family of X-pro-aminopeptidases that utilize a metal cofactor, and remove the N-terminal amino acid from peptides with a proline residue in the penultimate position. This protein has been shown to localize to the mitochondria of renal cells, and have a role in ciliary function. Mutations in this gene are associated with nephronophthisis-like nephropathy-1. Alternatively spliced transcript variants encoding different isoforms have been noted for this gene, however, expression of some of these isoforms in vivo is not known.