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Identification
HMDB Protein ID HMDBP13380
Secondary Accession Numbers None
Name Myeloblastin
Synonyms
  1. AGP7
  2. C-ANCA antigen
  3. Leukocyte proteinase 3
  4. Neutrophil proteinase 4
  5. P29
  6. Wegener autoantigen
  7. PR-3
  8. PR3
  9. NP-4
Gene Name PRTN3
Protein Type Unknown
Biological Properties
General Function Not Available
Specific Function Serine protease that degrades elastin, fibronectin, laminin, vitronectin, and collagen types I, III, and IV (in vitro) (PubMed:3198760, PubMed:2033050, PubMed:28240246). By cleaving and activating receptor F2RL1/PAR-2, enhances endothelial cell barrier function and thus vascular integrity during neutrophil transendothelial migration (PubMed:23202369). May play a role in neutrophil transendothelial migration, probably when associated with CD177 (PubMed:22266279).
Pathways Not Available
Reactions Not Available
GO Classification
Biological Process
negative regulation of phagocytosis
neutrophil degranulation
positive regulation of cell proliferation
phagocytosis
antimicrobial humoral response
cell-cell junction maintenance
collagen catabolic process
mature conventional dendritic cell differentiation
membrane protein ectodomain proteolysis
neutrophil extravasation
blood coagulation
proteolysis
cytokine-mediated signaling pathway
positive regulation of GTPase activity
Cellular Component
cytosol
extracellular vesicular exosome
plasma membrane
plasma membrane raft
extracellular region
azurophil granule lumen
extracellular space
intracellular membrane-bounded organelle
Molecular Function
receptor binding
serine-type peptidase activity
enzyme binding
serine-type endopeptidase activity
Cellular Location Not Available
Gene Properties
Chromosome Location Not Available
Locus Not Available
SNPs Not Available
Gene Sequence Not Available
Protein Properties
Number of Residues 256
Molecular Weight 27806.815
Theoretical pI 8.352
Pfam Domain Function
Signals
  • 1-25;
Transmembrane Regions Not Available
Protein Sequence Not Available
GenBank ID Protein Not Available
UniProtKB/Swiss-Prot ID P24158
UniProtKB/Swiss-Prot Entry Name PRTN3_HUMAN
PDB IDs
GenBank Gene ID Not Available
GeneCard ID Not Available
GenAtlas ID Not Available
HGNC ID Not Available
References
General References
  1. Gerhard DS, Wagner L, Feingold EA, Shenmen CM, Grouse LH, Schuler G, Klein SL, Old S, Rasooly R, Good P, Guyer M, Peck AM, Derge JG, Lipman D, Collins FS, Jang W, Sherry S, Feolo M, Misquitta L, Lee E, Rotmistrovsky K, Greenhut SF, Schaefer CF, Buetow K, Bonner TI, Haussler D, Kent J, Kiekhaus M, Furey T, Brent M, Prange C, Schreiber K, Shapiro N, Bhat NK, Hopkins RF, Hsie F, Driscoll T, Soares MB, Casavant TL, Scheetz TE, Brown-stein MJ, Usdin TB, Toshiyuki S, Carninci P, Piao Y, Dudekula DB, Ko MS, Kawakami K, Suzuki Y, Sugano S, Gruber CE, Smith MR, Simmons B, Moore T, Waterman R, Johnson SL, Ruan Y, Wei CL, Mathavan S, Gunaratne PH, Wu J, Garcia AM, Hulyk SW, Fuh E, Yuan Y, Sneed A, Kowis C, Hodgson A, Muzny DM, McPherson J, Gibbs RA, Fahey J, Helton E, Ketteman M, Madan A, Rodrigues S, Sanchez A, Whiting M, Madari A, Young AC, Wetherby KD, Granite SJ, Kwong PN, Brinkley CP, Pearson RL, Bouffard GG, Blakesly RW, Green ED, Dickson MC, Rodriguez AC, Grimwood J, Schmutz J, Myers RM, Butterfield YS, Griffith M, Griffith OL, Krzywinski MI, Liao N, Morin R, Palmquist D, Petrescu AS, Skalska U, Smailus DE, Stott JM, Schnerch A, Schein JE, Jones SJ, Holt RA, Baross A, Marra MA, Clifton S, Makowski KA, Bosak S, Malek J: The status, quality, and expansion of the NIH full-length cDNA project: the Mammalian Gene Collection (MGC). Genome Res. 2004 Oct;14(10B):2121-7. [PubMed:15489334 ]
  2. Grimwood J, Gordon LA, Olsen A, Terry A, Schmutz J, Lamerdin J, Hellsten U, Goodstein D, Couronne O, Tran-Gyamfi M, Aerts A, Altherr M, Ashworth L, Bajorek E, Black S, Branscomb E, Caenepeel S, Carrano A, Caoile C, Chan YM, Christensen M, Cleland CA, Copeland A, Dalin E, Dehal P, Denys M, Detter JC, Escobar J, Flowers D, Fotopulos D, Garcia C, Georgescu AM, Glavina T, Gomez M, Gonzales E, Groza M, Hammon N, Hawkins T, Haydu L, Ho I, Huang W, Israni S, Jett J, Kadner K, Kimball H, Kobayashi A, Larionov V, Leem SH, Lopez F, Lou Y, Lowry S, Malfatti S, Martinez D, McCready P, Medina C, Morgan J, Nelson K, Nolan M, Ovcharenko I, Pitluck S, Pollard M, Popkie AP, Predki P, Quan G, Ramirez L, Rash S, Retterer J, Rodriguez A, Rogers S, Salamov A, Salazar A, She X, Smith D, Slezak T, Solovyev V, Thayer N, Tice H, Tsai M, Ustaszewska A, Vo N, Wagner M, Wheeler J, Wu K, Xie G, Yang J, Dubchak I, Furey TS, DeJong P, Dickson M, Gordon D, Eichler EE, Pennacchio LA, Richardson P, Stubbs L, Rokhsar DS, Myers RM, Rubin EM, Lucas SM: The DNA sequence and biology of human chromosome 19. Nature. 2004 Apr 1;428(6982):529-35. [PubMed:15057824 ]
  3. Gabay JE, Scott RW, Campanelli D, Griffith J, Wilde C, Marra MN, Seeger M, Nathan CF: Antibiotic proteins of human polymorphonuclear leukocytes. Proc Natl Acad Sci U S A. 1989 Jul;86(14):5610-4. [PubMed:2501794 ]
  4. Gaskin G, Kendal H, Coulthart A, Turner N, Pusey CD: Use of proteinase 3 purified by reverse phase HPLC to detect autoantibodies in systemic vasculitis. J Immunol Methods. 1995 Mar 13;180(1):25-33. [PubMed:7897245 ]
  5. Duke-Cohan JS, Morimoto C, Rocker JA, Schlossman SF: A novel form of dipeptidylpeptidase IV found in human serum. Isolation, characterization, and comparison with T lymphocyte membrane dipeptidylpeptidase IV (CD26). J Biol Chem. 1995 Jun 9;270(23):14107-14. [PubMed:7539799 ]
  6. Zimmer M, Medcalf RL, Fink TM, Mattmann C, Lichter P, Jenne DE: 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. 1992 Sep 1;89(17):8215-9. [PubMed:1518849 ]
  7. Wilde CG, Snable JL, Griffith JE, Scott RW: Characterization of two azurphil granule proteases with active-site homology to neutrophil elastase. J Biol Chem. 1990 Feb 5;265(4):2038-41. [PubMed:2404977 ]
  8. Burkard TR, Planyavsky M, Kaupe I, Breitwieser FP, Burckstummer T, Bennett KL, Superti-Furga G, Colinge J: Initial characterization of the human central proteome. BMC Syst Biol. 2011 Jan 26;5:17. doi: 10.1186/1752-0509-5-17. [PubMed:21269460 ]
  9. Vaca Jacome AS, Rabilloud T, Schaeffer-Reiss C, Rompais M, Ayoub D, Lane L, Bairoch A, Van Dorsselaer A, Carapito C: N-terminome analysis of the human mitochondrial proteome. Proteomics. 2015 Jul;15(14):2519-24. doi: 10.1002/pmic.201400617. Epub 2015 Jun 8. [PubMed:25944712 ]
  10. Labbaye C, Musette P, Cayre YE: Wegener autoantigen and myeloblastin are encoded by a single mRNA. Proc Natl Acad Sci U S A. 1991 Oct 15;88(20):9253-6. doi: 10.1073/pnas.88.20.9253. [PubMed:1681549 ]
  11. Sturrock AB, Franklin KF, Rao G, Marshall BC, Rebentisch MB, Lemons RS, Hoidal JR: Structure, chromosomal assignment, and expression of the gene for proteinase-3. The Wegener's granulomatosis autoantigen. J Biol Chem. 1992 Oct 15;267(29):21193-9. [PubMed:1400430 ]
  12. Clave E, Molldrem J, Hensel N, Raptis A, Barrett AJ: Donor-recipient polymorphism of the proteinase 3 gene: a potential target for T-cell alloresponses to myeloid leukemia. J Immunother. 1999 Jan;22(1):1-6. doi: 10.1097/00002371-199901000-00001. [PubMed:9924693 ]
  13. Musette P, Labbaye C, Dorner MH, Cayre YE, Casanova JL, Kourilsky P: Wegener's autoantigen and leukemia. Blood. 1991 Mar 15;77(6):1398-9. [PubMed:2001463 ]
  14. Campanelli D, Melchior M, Fu Y, Nakata M, Shuman H, Nathan C, Gabay JE: Cloning of cDNA for proteinase 3: a serine protease, antibiotic, and autoantigen from human neutrophils. J Exp Med. 1990 Dec 1;172(6):1709-15. doi: 10.1084/jem.172.6.1709. [PubMed:2258701 ]
  15. Jenne DE, Tschopp J, Ludemann J, Utecht B, Gross WL: Wegener's autoantigen decoded. Nature. 1990 Aug 9;346(6284):520. doi: 10.1038/346520a0. [PubMed:2377228 ]
  16. Rao NV, Wehner NG, Marshall BC, Gray WR, Gray BH, Hoidal JR: Characterization of proteinase-3 (PR-3), a neutrophil serine proteinase. Structural and functional properties. J Biol Chem. 1991 May 25;266(15):9540-8. [PubMed:2033050 ]
  17. Ohlsson K, Linder C, Rosengren M: Monoclonal antibodies specific for neutrophil proteinase 4. Production and use for isolation of the enzyme. Biol Chem Hoppe Seyler. 1990 Jul;371(7):549-55. doi: 10.1515/bchm3.1990.371.2.549. [PubMed:2121162 ]
  18. Goldschmeding R, Dolman KM, van den Ende ME, van der Meer-Gerritsen CH, Sonnenberg A, von dem Borne AE: The relation of 29 kD C-ANCA antigen to proteinase 3. APMIS Suppl. 1990;19:26-7. doi: 10.1111/j.1600-0463.1990.tb05713.x. [PubMed:2285532 ]
  19. Niles JL, McCluskey RT, Ahmad MF, Arnaout MA: Wegener's granulomatosis autoantigen is a novel neutrophil serine proteinase. Blood. 1989 Nov 1;74(6):1888-93. [PubMed:2679910 ]
  20. Ludemann J, Utecht B, Gross WL: Anti-neutrophil cytoplasm antibodies in Wegener's granulomatosis recognize an elastinolytic enzyme. J Exp Med. 1990 Jan 1;171(1):357-62. doi: 10.1084/jem.171.1.357. [PubMed:1688612 ]
  21. Kao RC, Wehner NG, Skubitz KM, Gray BH, Hoidal JR: Proteinase 3. A distinct human polymorphonuclear leukocyte proteinase that produces emphysema in hamsters. J Clin Invest. 1988 Dec;82(6):1963-73. doi: 10.1172/JCI113816. [PubMed:3198760 ]
  22. von Vietinghoff S, Tunnemann G, Eulenberg C, Wellner M, Cristina Cardoso M, Luft FC, Kettritz R: NB1 mediates surface expression of the ANCA antigen proteinase 3 on human neutrophils. Blood. 2007 May 15;109(10):4487-93. doi: 10.1182/blood-2006-10-055327. Epub 2007 Jan 23. [PubMed:17244676 ]
  23. von Vietinghoff S, Eulenberg C, Wellner M, Luft FC, Kettritz R: Neutrophil surface presentation of the anti-neutrophil cytoplasmic antibody-antigen proteinase 3 depends on N-terminal processing. Clin Exp Immunol. 2008 Jun;152(3):508-16. doi: 10.1111/j.1365-2249.2008.03663.x. [PubMed:18462208 ]
  24. Jerke U, Rolle S, Dittmar G, Bayat B, Santoso S, Sporbert A, Luft F, Kettritz R: Complement receptor Mac-1 is an adaptor for NB1 (CD177)-mediated PR3-ANCA neutrophil activation. J Biol Chem. 2011 Mar 4;286(9):7070-81. doi: 10.1074/jbc.M110.171256. Epub 2010 Dec 30. [PubMed:21193407 ]
  25. Kuckleburg CJ, Newman PJ: Neutrophil proteinase 3 acts on protease-activated receptor-2 to enhance vascular endothelial cell barrier function. Arterioscler Thromb Vasc Biol. 2013 Feb;33(2):275-84. doi: 10.1161/ATVBAHA.112.300474. Epub 2012 Nov 29. [PubMed:23202369 ]
  26. Jerke U, Marino SF, Daumke O, Kettritz R: Characterization of the CD177 interaction with the ANCA antigen proteinase 3. Sci Rep. 2017 Feb 27;7:43328. doi: 10.1038/srep43328. [PubMed:28240246 ]
  27. Choi YJ, Kim S, Choi Y, Nielsen TB, Yan J, Lu A, Ruan J, Lee HR, Wu H, Spellberg B, Jung JU: SERPINB1-mediated checkpoint of inflammatory caspase activation. Nat Immunol. 2019 Mar;20(3):276-287. doi: 10.1038/s41590-018-0303-z. Epub 2019 Jan 28. [PubMed:30692621 ]
  28. Fujinaga M, Chernaia MM, Halenbeck R, Koths K, James MN: The crystal structure of PR3, a neutrophil serine proteinase antigen of Wegener's granulomatosis antibodies. J Mol Biol. 1996 Aug 16;261(2):267-78. doi: 10.1006/jmbi.1996.0458. [PubMed:8757293 ]