Recombinant Human Myeloblastin (PRTN3)

1. PR3 is involved in triggering inflammatory pathways, disrupting cellular signalling, degrading key structural proteins, and pathogen response.PR3 dysfunction may play a critical role in many processes central to the pathophysiology of COPD and other chronic neutrophilic human diseases. PMID: 30236095
2. Characterization of the CD177 interaction with the ANCA antigen proteinase 3 responsible for autoimmune diseases has been reported. PMID: 28240246
3. Our results demonstrate that in the early stage of sepsis, JMJD3 contributes to high levels of neutrophil mPR3 expression and thereby to the production of the inflammatory cytokine IL-1beta PMID: 29621735
4. A few peptides bound to proteinase 3 (PR3) hydrophobic pockets and inhibited PR3 binding to lipids, while the (KFF)3K d-peptide inshowed a significant affinity for the lipids. PMID: 29132840
5. Studies indicate that the premature activation of proteinase 3 (PR3) would seriously harm the cell by its protease activity. PMID: 27559009
6. this study shows that transgenic mice expressing human Proteinase 3 exhibit sustained neutrophil-associated peritonitis PMID: 29079698
7. unique structural and functional characteristics of PR3 might be key contributors to the systemic inflammation and to the immune dysregulation observed in granulomatosis with polyangiitis. PMID: 28546501
8. Our findings demonstrate how RAGE-PR3 interactions between human prostate cancer cells and the bone marrow microenvironment mediate bone metastasis during prostate cancer progression PMID: 28428279
9. PRTN3 variants are associated with ANCA-associated vasculitis risk. PMID: 28029757
10. Clinical manifestations varied ANCA-associated vasculitis categories, and neither MPO-ANCA nor PR3-ANCA significantly affected relapse of AAV. PMID: 28339364
11. Changes in the DNA methylation status of the PRTN3 promoter may predict the likelihood of stable remission and explain autoantigen gene regulation PMID: 27821628
12. Study shows that MPO and PRTN3 in neutrophils of Anti-neutrophil cytoplasmic autoantibody (ANCA)-associated vasculitis (AAV) patients with active disease have a distinct pattern of histone modifications, which implicates epigenetic mechanisms in regulating expression of autoantigen genes and suggests that the epigenome may be involved in AAV pathogenesis. PMID: 27752292
13. The levels of both NE and PR3 correlated with absolute neutrophil counts and are reduced in type 1 diabetes mellitus patients. PMID: 26939803
14. Report prognostic value of proteinase-3-antineutrophil cytoplasmic antibody in patients with idiopathic interstitial pneumonias. PMID: 26873743
15. Molecular simulations suggest that PR3 interacts with phosphatidylserine via a small number of amino acids, which engage in long lasting interactions with the lipid heads. As phosphatidylserine is a major component of microvesicles (MVs), this study also examined the consequences of this interaction on MV production and function. PMID: 26961880
16. miR-634 mimics induced a proinflammatory phenotype in monocyte-derived macrophages, with enhanced expression and release of ADAM17 and IL-6 in Proteinase-3 ANCA-Associated Vasculitis PMID: 25788529
17. The PR3-induced microenvironment facilitated recruitment of inflammatory cells, such as macrophages, plasmacytoid DCs (pDCs), and neutrophils, which were observed in close proximity within granulomatous lesions in the lungs of GPA patients. PMID: 26436651
18. ACPA negative rheumatoid arthritis shares the genetic susceptibility loci with ACPA positive disease. PMID: 25927497
19. Data suggest that targeting proteinase-3 (PR3) specific effector memory cells, to prevent relapse, and instituting anti IL-17 therapy, or modulating Tregs could be newer forms of therapy for granulomatosis with polyangiitis (GPA). PMID: 25461407
20. Anti-PR3 autoantibodies not detected in sera of patients with pulmonary tuberculosis. PMID: 24719228
21. these results highlight a potentially important role for proteinase 3 in emphysema. PMID: 25416382
22. The data shows that the neutrophil protease PR3 is a direct modulator of human platelets and causes shape change through activation of the Rho/Rho kinase and Ca(2+) signaling pathways. PMID: 24993595
23. These in vivo data provide, for the first time, compelling evidence of the collateral involvement of cathepsin G, NE, and proteinase 3 in cigarette smoke-induced tissue damage and emphysema PMID: 24929239
24. Proteinase 3-mediated caspase-3 activation controls neutrophil spontaneous death PMID: 25180606
25. Elevated levels of GCF proteinase 3 in CP, G-AgP, and gingivitis might suggest that proteinase 3 plays a role during inflammatory periodontal events in host response. PMID: 24949444
26. Circulating protein levels and enzymatic activity of PRTN3 are markedly elevated in patients with type 1 diabetes mellitus, and are correlated with increased autoantibodies against beta-cell antigens. PMID: 25092677
27. It reports the synthesis and enzymatic studies on a new proteinase 3 intermolecular quenched substrate with enhanced selectivity over neutrophil elastase. PMID: 23911525
28. The presence of ANCA in the bile of patients with primary sclerosing cholangitis (PSC) is a novel finding and highly suggestive of PSC. Biliary IgG ANCA correlates with the severity of bile duct strictures. PMID: 23957616
29. Lateral flow assay for the determination of IgG-autoantibodies to Pr3. PMID: 24291125
30. Proteinase 3-anti-neutrophil cytoplasmic antibody (ANCA) serum and cerebrospinal fluid (CSF) levels are used as a marker for the more severe form of hypertrophic pachymeningitis. PMID: 24271323
31. Neutrophil elastase and proteinase-3 trigger G protein-biased signaling through proteinase-activated receptor-1 (PAR1). PMID: 24052258
32. Proteinase 3 may play a greater role in alpha-1-antitrypsin deficiency and COPD PMID: 22936713
33. Changes in proteinase 3 anti-neutrophil cytoplasm autoantibody levels in early systemic granulomatosis with polyangiitis (Wegener's) may reflect treatment rather than disease activity. PMID: 23380137
34. Neutrophil proteinase 3 plays a significant role in promoting vascular integrity by signaling through endothelial cell PAR-2. PMID: 23202369
35. PAR signaling and serine protease-induced alterations in endothelial function modulate glomerular inflammation via parallel but independent pathways PMID: 22952809
36. Some anti-PR3 responses are highly variable across donors. PMID: 22791638
37. Membrane PR3 expressed on apoptotic neutrophils might amplify inflammation by affecting the anti-inflammatory "reprogramming" of macrophages. PMID: 22844112
38. Early plasma exchange in addition to cyclophosphamide/glucocorticoid treatment improves outcome in PR3-ANCA-positive renal vasculitis. PMID: 22510451
39. PR3 expression and activity are significantly increased on transmigrating neutrophils; PR3 and NB1/CD177 interactions may play a role in aiding neutrophil transmigration. PMID: 22266279
40. Proteinase 3 carries small unusual carbohydrates and associates with alphalpha-defensins. PMID: 22138257
41. the dual functions (activation/termination) of PR3 in IL-33 biological activity. PMID: 22270365
42. the activity of myeloblastin was significantly altered in the lung adenocarcinoma biopsies harboring a KRAS gene mutation. PMID: 22210048
43. membrane-bound PR3 acts as a non-opsonic phagocytosis receptor for bacteria probably by activating PAR2 in neutrophils PMID: 21700341
44. Suggest that in Wegener's granulomatosis and Churg-Strauss syndrome the cytokine response of the total CD4+ T-cell population and PR3-specific cells is influenced by the underlying disorder. PMID: 21470489
45. neutrophil elastase (NE) and proteinase-3 are capable of almost complete degradation of hemoglobin in vitro PMID: 21193404
46. pivotal role of the NB1-Mac-1 receptor interaction for PR3-ANCA-mediated neutrophil activation PMID: 21193407
47. healthy individuals have masked circulating, noncross-reactive, antigen-specific natural autoantibodies against MPO, PR3, and GBM in their serum and IgG fraction PMID: 20592714
48. The trafficking of precursor NE (proNE) and precursor PR3 (proPR3), was compared. PMID: 20828556
49. cANCAs from Wegener's granulomatosis patients at least in part recognize similar surface structures as do mouse monoclonal antibodies and compete with the binding of alpha1-protease inhibitor to PR3. PMID: 20530264
50. The increased membrane expression of PR3 found in ANCA-associated systemic vasculitis is not linked directly to circulating PR3 or PR3 gene transcription, but is dependent upon CD177 expression and correlated with the transcription of the CD177 gene. PMID: 20491791

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Is the major autoantigen in anti-neutrophil cytoplasmic autoantibody (ANCA)-associated vasculitis (Wegener's granulomatosis) (PubMed:2377228, PubMed:2679910). This complex, systemic disease is characterized by granulomatous inflammation with necrotizing lesions in the respiratory tract, glomerulonephritis, vasculitis, and anti-neutrophil cytoplasmatic autoantibodies detected in patient sera (PubMed:2377228, PubMed:2679910). PRTN3 causes emphysema when administered by tracheal insufflation to hamsters (PubMed:3198760).

Cytoplasmic granule. Secreted. Cell membrane; Peripheral membrane protein; Extracellular side. Membrane raft; Peripheral membrane protein; Extracellular side.

Peptidase S1 family, Elastase subfamily

Expressed in polymorphonuclear leukocytes (at protein level). Expressed in neutrophils (at protein level). Expressed in differentiating neutrophils.

HGNC: 9495

OMIM: 177020

KEGG: hsa:5657

STRING: 9606.ENSP00000234347

UniGene: Hs.928