Proteinase 3

Proteinase 3, also known as PRTN3, is an enzyme that in humans is encoded by the PRTN3 gene.

PRTN3
Available structures PDB Ortholog search: PDBe RCSB List of PDB id codes 1FUJ
Identifiers
Aliases	PRTN3, ACPA, AGP7, C-ANCA, CANCA, MBN, MBT, NP-4, NP4, P29, PR-3, PR3, proteinase 3
External IDs	OMIM: 177020; MGI: 893580; HomoloGene: 120934; GeneCards: PRTN3; OMA:PRTN3 - orthologs
Gene location (Human) Chr. Chromosome 19 (human) Band 19p13.3 Start 840,999 bp End 848,175 bp
Gene location (Mouse) Chr. Chromosome 10 (mouse) Band 10 C1|10 39.72 cM Start 79,710,310 bp End 79,719,008 bp
RNA expression pattern Bgee Human Mouse (ortholog) Top expressed in bone marrow bone marrow cells spleen blood monocyte granulocyte right lung superior frontal gyrus right frontal lobe dorsolateral prefrontal cortex Top expressed in tibiofemoral joint granulocyte fetal liver hematopoietic progenitor cell body of femur bone marrow ankle joint blastocyst embryo human fetus embryo More reference expression data BioGPS More reference expression data
Gene ontology Molecular function enzyme binding peptidase activity serine-type peptidase activity hydrolase activity protein binding serine-type endopeptidase activity signaling receptor binding Cellular component extracellular matrix plasma membrane extracellular exosome cytosol extracellular region extracellular space azurophil granule lumen plasma membrane raft membrane membrane raft collagen-containing extracellular matrix Biological process collagen catabolic process blood coagulation negative regulation of phagocytosis mature conventional dendritic cell differentiation positive regulation of cell population proliferation antimicrobial humoral response neutrophil degranulation proteolysis membrane protein ectodomain proteolysis positive regulation of GTPase activity cell-cell junction maintenance neutrophil extravasation phagocytosis cytokine-mediated signaling pathway Sources:Amigo / QuickGO
Orthologs Species Human Mouse Entrez 5657 19152 Ensembl ENSG00000277804 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: 79.71 – 79.72 Mb PubMed search
Wikidata
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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 target 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.

Further reading

• Martin KR, Witko-Sarsat V (September 2017). "Proteinase 3: the odd one out that became an autoantigen". Journal of Leukocyte Biology. 102 (3): 689–698. doi:10.1189/jlb.3MR0217-069R. PMID 28546501.
• van der Helm-van Mil AH, Huizinga TW, de Vries RR, Toes RE (June 2007). "Emerging patterns of risk factor make-up enable subclassification of rheumatoid arthritis". Arthritis and Rheumatism. 56 (6): 1728–35. doi:10.1002/art.22716. PMID 17534941.
• Kettritz R (September 2016). "Neutral serine proteases of neutrophils". Immunological Reviews. 273 (1): 232–48. doi:10.1111/imr.12441. PMID 27558338. S2CID 29724551.</ref>
• Zimmer M, Medcalf RL, Fink TM, Mattmann C, Lichter P, Jenne DE (September 1992). "Three human elastase-like genes coordinately expressed in the myelomonocyte lineage are organized as a single genetic locus on 19pter". Proceedings of the National Academy of Sciences of the United States of America. 89 (17): 8215–9. Bibcode:1992PNAS...89.8215Z. doi:10.1073/pnas.89.17.8215. PMC 49888. PMID 1518849.
• Cornec D, Cornec-Le Gall E, Fervenza FC, Specks U (October 2016). "ANCA-associated vasculitis - clinical utility of using ANCA specificity to classify patients". Nature Reviews. Rheumatology. 12 (10): 570–9. doi:10.1038/nrrheum.2016.123. PMID 27464484. S2CID 22205737.
• Rao NV, Wehner NG, Marshall BC, Gray WR, Gray BH, Hoidal JR (May 1991). "Characterization of proteinase-3 (PR-3), a neutrophil serine proteinase. Structural and functional properties". The Journal of Biological Chemistry. 266 (15): 9540–8. doi:10.1016/S0021-9258(18)92854-1. PMID 2033050.
• Ohlsson K, Linder C, Rosengren M (July 1990). "Monoclonal antibodies specific for neutrophil proteinase 4. Production and use for isolation of the enzyme". Biological Chemistry Hoppe-Seyler. 371 (7): 549–55. doi:10.1515/bchm3.1990.371.2.549. PMID 2121162.
• 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". The Journal of Experimental Medicine. 172 (6): 1709–15. doi:10.1084/jem.172.6.1709. PMC 2188764. PMID 2258701.
• Goldschmeding R, Dolman KM, van den Ende ME, van der Meer-Gerritsen CH, Sonnenberg A, von dem Borne AE (1991). "The relation of 29 kD C-ANCA antigen to proteinase 3". APMIS. 98 (S19): 26–7. doi:10.1111/j.1600-0463.1990.tb05713.x. PMID 2285532. S2CID 37665338.
• Wilde CG, Snable JL, Griffith JE, Scott RW (February 1990). "Characterization of two azurphil granule proteases with active-site homology to neutrophil elastase". The Journal of Biological Chemistry. 265 (4): 2038–41. doi:10.1016/S0021-9258(19)39936-3. PMID 2404977.
• Gabay JE, Scott RW, Campanelli D, Griffith J, Wilde C, Marra MN, Seeger M, Nathan CF (July 1989). "Antibiotic proteins of human polymorphonuclear leukocytes". Proceedings of the National Academy of Sciences of the United States of America. 86 (14): 5610–4. Bibcode:1989PNAS...86.5610G. doi:10.1073/pnas.86.14.5610. PMC 297672. PMID 2501794.
• Bories D, Raynal MC, Solomon DH, Darzynkiewicz Z, Cayre YE (December 1989). "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.
• Pontremoli S, Melloni E, Michetti M, Sacco O, Sparatore B, Salamino F, Damiani G, Horecker BL (March 1986). "Cytolytic effects of neutrophils: role for a membrane-bound neutral proteinase". Proceedings of the National Academy of Sciences of the United States of America. 83 (6): 1685–9. Bibcode:1986PNAS...83.1685P. doi:10.1073/pnas.83.6.1685. PMC 323148. PMID 3513185.
• Sugimori T, Cooley J, Hoidal JR, Remold-O'Donnell E (September 1995). "Inhibitory properties of recombinant human monocyte/neutrophil elastase inhibitor". American Journal of Respiratory Cell and Molecular Biology. 13 (3): 314–22. doi:10.1165/ajrcmb.13.3.7654387. PMID 7654387.
• Brunini F, Page TH, Gallieni M, Pusey CD (November 2016). "The role of monocytes in ANCA-associated vasculitides" (PDF). Autoimmunity Reviews. 15 (11): 1046–1053. doi:10.1016/j.autrev.2016.07.031. hdl:10044/1/39150. PMID 27491570.

External links

• Proteinase+3 at the U.S. National Library of Medicine Medical Subject Headings (MeSH)