| Proteinase 3 (serine proteinase, neutrophil, Wegener granulomatosis autoantigen)
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| PDB rendering based on 1fuj.
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| Available structures: 1fuj
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| Identifiers
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| Symbol(s)
| PRTN3; ACPA; AGP7; C-ANCA; MBT; P29; PR-3
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| External IDs
| OMIM: 177020 MGI: 893580 Homologene: 20615
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| Gene Ontology
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| Molecular Function:
| • serine-type endopeptidase activity
• protein binding
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| Cellular Component:
| • cellular_component
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| Biological Process:
| • proteolysis
• positive regulation of cell proliferation
• collagen catabolic process
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| RNA expression pattern
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More reference expression data
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| Orthologs
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| Human
| Mouse
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| Entrez
| 5657
| 19152
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| Ensembl
| ENSG00000196415
| ENSMUSG00000057729
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| Uniprot
| P24158
| Q61096
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| Refseq
| NM_002777 (mRNA)
NP_002768 (protein)
| NM_011178 (mRNA)
NP_035308 (protein)
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| Location
| Chr 19: 0.79 - 0.8 Mb
| Chr 10: 79.28 - 79.29 Mb
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| Pubmed search
| [1]
| [2]
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Proteinase 3 is a serine protease enzyme expressed mainly in neutrophil granulocytes. Its exact role in the function of the neutrophil is unknown. It is also the epitope of anti-neutrophil cytoplasmic antibodies (ANCAs) of the cANCA (cytoplasmic subtype) class, a type of antibody frequently found in the disease Wegener's granulomatosis.
Additional recommended knowledge
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. 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. 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. 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. 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. 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. 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. 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. 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. 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. PMID 8261669.
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