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> XPNPEP2 XPNPEP2
Aminopeptidase P (APP) is a hydrolase specific for N-terminal imido bonds, which are common to several collagen degradation products, neuropeptides, vasoactive peptides, and cytokines. A membrane-bound and soluble form of this enzyme (XPNPEP2) have been identified as products of two separate genes. XPNPEP2, the X-linked gene that encodes membranous aminopeptidase P (APP), has been reported to associate with APP activity. The membrane aminopeptidase P (XPNPEP2) is largely limited in distribution to endothelia and brush border epithelia. APP and XPNPEP2 contain homologous blocks of sequence common to members of the "pita bread-fold" protein family, of which Escherichia coli methionine aminopeptidase is the prototype. The C-2399A variant in XPNPEP2 is associated with reduced APP activity and a higher incidence of AE-ACEi. XPNPEP2 mRNA was detected in fibroblasts that carry the translocation, suggesting that this gene at least partially escapes X inactivation. XPNPEP2 is a candidate gene for premature ovarian failure (POF).
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XPNPEP2 cDNA Clones
XPNPEP2 Related Areas
Enzyme>>Protease & Regulator>>Other Proteases>>XPNPEP2
XPNPEP2 Related Pathways
XPNPEP2 Alternative Names
XPNPEP2, RP4-753P9.2, APP2 [Homo sapiens]
Xpnpep2, RP23-351G20.1, 9030008G12Rik, mAPP [Mus musculus]
Summaries for XPNPEP2
Entrez Gene summary for XPNPEP2:
Aminopeptidase P is a hydrolase specific for N-terminal imido bonds, which are common to several collagen degradation products, neuropeptides, vasoactive peptides, and cytokines. Structurally, the enzyme is a member of the 'pita bread fold' family and occurs in mammalian tissues in both soluble and GPI-anchored membrane-bound forms. A membrane-bound and soluble form of this enzyme have been identified as products of two separate genes. [provided by RefSeq, Jul 2008]
Wikipedia summary for XPNPEP2:
Xaa-Pro aminopeptidase 2 is an enzyme that in humans is encoded by the XPNPEP2 gene.
Aminopeptidase P is a hydrolase specific for N-terminal imido bonds, which are common to several collagen degradation products, neuropeptides, vasoactive peptides, and cytokines. Structurally, the enzyme is a member of the 'pita bread fold' family and occurs in mammalian tissues in both soluble and GPI-anchored membrane-bound forms. A membrane-bound and soluble form of this enzyme have been identified as products of two separate genes.
Human XPNPEP2 Protein General Information
| Protein names |
Xaa-Pro aminopeptidase 2 |
| Sequence length |
674 AA. |
| Domain |
Signal |
| Sequence similarities: |
Belongs to the peptidase M24B family. |
| Post-translational modification: |
Heavily glycosylated. |
| Subunit structure |
Homotrimer. |
| Subcellular location: | Cell membrane; Lipid-anchor › GPI-anchor. |
| Tissue specificity |
Expressed in kidney, lung, heart, placenta, liver, small intestine and colon. No expression in brain, skeletal muscle, pancreas, spleen, thymus, prostate, testis and ovary. |
| Cofactor: | Binds 2 manganese ions per subunit |
| Catalytic activity |
Release of any N-terminal amino acid, including proline, that is linked to proline, even from a dipeptide or tripeptide. |
General information above from UniProt
Function for XPNPEP2 Protein
UniProtKB:
XPNPEP2 is a metalloprotease that may play a role in the inflammatory process and other reactions produced in response to injury or infection. XPNPEP2 may also play a role in the metabolism of the vasodilator bradykinin.
Genatlas:
- XPNPEP2 is X-prolyl aminopeptidase, hydrolyzing N terminal imido (Xaa-Pro) bonds
- XPNPEP2 presumably functions in the degradation of both dietary and filtered peptides by intestinal and renal epithelial brush border membranes, respectively
- XPNPEP2 may have unrecognized roles in cell trafficking and in immune function
- XPNPEP2 coinactivates vasodilatator bradykinin in the lung with angiotensin converting enzyme
- XPNPEP2 can hydrolyze bradykinin containing a penultimate proline residue
Homology for human XPNPEP2
- homolog to E.coli methionine amidopeptidase
Phenotype Information for XPNPEP2
| Gene/Locus | Phenotype |
| XPNPEP2 | {Angioedema induced by ACE inhibitors, susceptibility to} |
Phenotype Information for XPNPEP2 from OMIM (Online Mendelian Inheritance in Man)

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