Proteinase 3

PRTN3
Available structures
PDBOrtholog search: PDBe RCSB
Identifiers
AliasesPRTN3, ACPA, AGP7, C-ANCA, CANCA, MBN, MBT, NP-4, NP4, P29, PR-3, PR3, proteinase 3
External IDsOMIM: 177020 MGI: 893580 HomoloGene: 120934 GeneCards: PRTN3
Gene location (Human)
Chr.Chromosome 19 (human)[1]
Band19p13.3Start840,960 bp[1]
End848,175 bp[1]
RNA expression pattern
More reference expression data
Orthologs
SpeciesHumanMouse
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 MbChr 10: 79.87 – 79.88 Mb
PubMed search[3][4]
Wikidata
View/Edit HumanView/Edit Mouse

Proteinase 3 also known as PRTN3 is an enzyme that in humans is encoded by the PRTN3 gene.[5]

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 (formerly known as "Wegener's granulomatosis").[6]

References

  1. 1 2 3 ENSG00000196415 GRCh38: Ensembl release 89: ENSG00000277804, ENSG00000196415 - Ensembl, May 2017
  2. 1 2 3 GRCm38: Ensembl release 89: ENSMUSG00000057729 - Ensembl, May 2017
  3. ↑ "Human PubMed Reference:".
  4. ↑ "Mouse PubMed Reference:".
  5. ↑ 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.
  6. ↑ 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.
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