Proteinase 3

Proteinase 3
PDB rendering based on 1fuj.
Available structures PDB Ortholog search: PDBe, RCSB List of PDB id codes 1FUJ​
Identifiers
Symbols	PRTN3 ; ACPA; AGP7; C-ANCA; CANCA; MBN; MBT; NP-4; NP4; P29; PR-3; PR3
External IDs	OMIM: 177020 MGI: 893580 HomoloGene: 120934 ChEMBL: 3900 GeneCards: PRTN3 Gene
EC number	3.4.21.76
Gene ontology Molecular function • serine-type endopeptidase activity • serine-type peptidase activity • enzyme binding Cellular component • extracellular space • cytosol • plasma membrane • extracellular matrix • extracellular exosome Biological process • proteolysis • blood coagulation • positive regulation of cell proliferation • collagen catabolic process • negative regulation of phagocytosis • mature conventional dendritic cell differentiation Sources: Amigo / QuickGO
RNA expression pattern
More reference expression data
Orthologs
Species	Human	Mouse
Entrez	5657	19152
Ensembl	ENSG00000196415	ENSMUSG00000057729
UniProt	P24158	Q61096
RefSeq (mRNA)	NM_002777	NM_011178
RefSeq (protein)	NP_002768	NP_035308
Location (UCSC)	Chr 19: 0.84 – 0.85 Mb	Chr 10: 79.87 – 79.88 Mb
PubMed search

Proteinase 3 also known as PRTN3 is an enzyme that in humans is encoded by the PRTN3 gene.^[1]

Function

PRTN3 is a serine protease enzyme expressed mainly in neutrophil granulocytes. Its exact role in the function of the neutrophil is unknown, but, in human neutrophils, proteinase 3 contributes to the proteolytic generation of antimicrobial peptides. It is also the epitope of anti-neutrophil cytoplasmic antibodies (ANCAs) of the c-ANCA (cytoplasmic subtype) class, a type of antibody frequently found in the disease granulomatosis with polyangiitis (formerly known as "Wegener's granulomatosis").^[2]

References

1. Campanelli D, Melchior M, Fu Y, Nakata M, Shuman H, Nathan C, Gabay JE (December 1990). "Cloning of cDNA for proteinase 3: a serine protease, antibiotic, and autoantigen from human neutrophils". J. Exp. Med. 172 (6): 1709–15. doi:10.1084/jem.172.6.1709. PMC 2188764. PMID 2258701.
2. van der Geld YM, Limburg PC, Kallenberg CG (February 2001). "Proteinase 3, Wegener's autoantigen: from gene to antigen". J. Leukoc. Biol. 69 (2): 177–90. PMID 11272267.

Further reading

• Watorek E, Boratyńska M, Klinger M (2004). "Wegener's granulomatosis--autoimmunity to neutrophil proteinase 3". Arch. Immunol. Ther. Exp. (Warsz.) 51 (3): 157–67. PMID 12894870.
• van der Helm-van Mil AH, Huizinga TW, de Vries RR, Toes RE (2007). "Emerging patterns of risk factor make-up enable subclassification of rheumatoid arthritis". Arthritis Rheum. 56 (6): 1728–35. doi:10.1002/art.22716. PMID 17534941.
• Sturrock AB, Franklin KF, Rao G, et al. (1992). "Structure, chromosomal assignment, and expression of the gene for proteinase-3. The Wegener's granulomatosis autoantigen". J. Biol. Chem. 267 (29): 21193–9. PMID 1400430.
• Zimmer M, Medcalf RL, Fink TM, et al. (1992). "Three human elastase-like genes coordinately expressed in the myelomonocyte lineage are organized as a single genetic locus on 19pter". Proc. Natl. Acad. Sci. U.S.A. 89 (17): 8215–9. doi:10.1073/pnas.89.17.8215. PMC 49888. PMID 1518849.
• Labbaye C, Musette P, Cayre YE (1991). "Wegener autoantigen and myeloblastin are encoded by a single mRNA". Proc. Natl. Acad. Sci. U.S.A. 88 (20): 9253–6. doi:10.1073/pnas.88.20.9253. PMC 52692. PMID 1681549.
• Lüdemann J, Utecht B, Gross WL (1990). "Anti-neutrophil cytoplasm antibodies in Wegener's granulomatosis recognize an elastinolytic enzyme". J. Exp. Med. 171 (1): 357–62. doi:10.1084/jem.171.1.357. PMC 2187669. PMID 1688612.
• Musette P, Labbaye C, Dorner MH, et al. (1991). "Wegener's autoantigen and leukemia". Blood 77 (6): 1398–9. PMID 2001463.
• Rao NV, Wehner NG, Marshall BC, et al. (1991). "Characterization of proteinase-3 (PR-3), a neutrophil serine proteinase. Structural and functional properties". J. Biol. Chem. 266 (15): 9540–8. PMID 2033050.
• Ohlsson K, Linder C, Rosengren M (1990). "Monoclonal antibodies specific for neutrophil proteinase 4. Production and use for isolation of the enzyme". Biol. Chem. Hoppe-Seyler 371 (7): 549–55. doi:10.1515/bchm3.1990.371.2.549. PMID 2121162.
• Gupta SK, Niles JL, McCluskey RT, Arnaout MA (1990). "Identity of Wegener's autoantigen (p29) with proteinase 3 and myeloblastin". Blood 76 (10): 2162. PMID 2242436.
• Campanelli D, Melchior M, Fu Y, et al. (1991). "Cloning of cDNA for proteinase 3: a serine protease, antibiotic, and autoantigen from human neutrophils". J. Exp. Med. 172 (6): 1709–15. doi:10.1084/jem.172.6.1709. PMC 2188764. PMID 2258701.
• Goldschmeding R, Dolman KM, van den Ende ME, et al. (1991). "The relation of 29 kD C-ANCA antigen to proteinase 3". APMIS Suppl. 19: 26–7. PMID 2285532.
• Jenne DE, Tschopp J, Lüdemann J, et al. (1990). "Wegener's autoantigen decoded". Nature 346 (6284): 520. doi:10.1038/346520a0. PMID 2377228.
• Wilde CG, Snable JL, Griffith JE, Scott RW (1990). "Characterization of two azurphil granule proteases with active-site homology to neutrophil elastase". J. Biol. Chem. 265 (4): 2038–41. PMID 2404977.
• Gabay JE, Scott RW, Campanelli D, et al. (1989). "Antibiotic proteins of human polymorphonuclear leukocytes". Proc. Natl. Acad. Sci. U.S.A. 86 (14): 5610–4. doi:10.1073/pnas.86.14.5610. PMC 297672. PMID 2501794.
• Bories D, Raynal MC, Solomon DH, et al. (1990). "Down-regulation of a serine protease, myeloblastin, causes growth arrest and differentiation of promyelocytic leukemia cells". Cell 59 (6): 959–68. doi:10.1016/0092-8674(89)90752-6. PMID 2598267.
• Niles JL, McCluskey RT, Ahmad MF, Arnaout MA (1989). "Wegener's granulomatosis autoantigen is a novel neutrophil serine proteinase". Blood 74 (6): 1888–93. PMID 2679910.
• Pontremoli S, Melloni E, Michetti M, et al. (1986). "Cytolytic effects of neutrophils: role for a membrane-bound neutral proteinase". Proc. Natl. Acad. Sci. U.S.A. 83 (6): 1685–9. doi:10.1073/pnas.83.6.1685. PMC 323148. PMID 3513185.
• Sugimori T, Cooley J, Hoidal JR, Remold-O'Donnell E (1995). "Inhibitory properties of recombinant human monocyte/neutrophil elastase inhibitor". Am. J. Respir. Cell Mol. Biol. 13 (3): 314–22. doi:10.1165/ajrcmb.13.3.7654387. PMID 7654387.
• Muller-Bérat N, Minowada J, Tsuji-Takayama K, et al. (1994). "The phylogeny of proteinase 3/myeloblastin, the autoantigen in Wegener's granulomatosis, and myeloperoxidase as shown by immunohistochemical studies on human leukemic cell lines". Clin. Immunol. Immunopathol. 70 (1): 51–9. doi:10.1006/clin.1994.1010. PMID 8261669.

External links

• Proteinase 3 at the US National Library of Medicine Medical Subject Headings (MeSH)