Summary: A polymorphonuclear leukocyte-derived serine protease that degrades proteins such as ELASTIN; FIBRONECTIN; LAMININ; VITRONECTIN; and COLLAGEN. It is named for its ability to control myeloid cell growth and differentiation.

Top Publications

  1. Uh M, McCormick I, Kelsall J. Positive cytoplasmic antineutrophil cytoplasmic antigen with PR3 specificity glomerulonephritis in a patient with subacute bacterial endocarditis. J Rheumatol. 2011;38:1527-8 pubmed publisher
  2. Eick S, Puklo M, Adamowicz K, Kantyka T, Hiemstra P, Stennicke H, et al. Lack of cathelicidin processing in Papillon-Lefèvre syndrome patients reveals essential role of LL-37 in periodontal homeostasis. Orphanet J Rare Dis. 2014;9:148 pubmed publisher
    ..actinomycetemcomitans and the development of severe periodontal disease. ..
  3. Millet A, Martin K, Bonnefoy F, Saas P, Mocek J, Alkan M, et al. Proteinase 3 on apoptotic cells disrupts immune silencing in autoimmune vasculitis. J Clin Invest. 2015;125:4107-21 pubmed publisher
    ..These results reveal that PR3 disrupts immune silencing associated with clearance of apoptotic neutrophils and provide insight into how PR3 and PR3-targeting ANCAs promote GPA pathophysiology. ..
  4. Sethi S, Zand L, De Vriese A, Specks U, Vrana J, Kanwar S, et al. Complement activation in pauci-immune necrotizing and crescentic glomerulonephritis: results of a proteomic analysis. Nephrol Dial Transplant. 2017;32:i139-i145 pubmed publisher
  5. Kolonin M, Sergeeva A, Staquicini D, Smith T, Tarleton C, Molldrem J, et al. Interaction between Tumor Cell Surface Receptor RAGE and Proteinase 3 Mediates Prostate Cancer Metastasis to Bone. Cancer Res. 2017;77:3144-3150 pubmed publisher
    ..i>Cancer Res; 77(12); 3144-50. ©2017 AACR. ..
  6. Bossuyt X, Rasmussen N, van Paassen P, Hellmich B, Baslund B, Vermeersch P, et al. A multicentre study to improve clinical interpretation of proteinase-3 and myeloperoxidase anti-neutrophil cytoplasmic antibodies. Rheumatology (Oxford). 2017;56:1533-1541 pubmed publisher
    ..Higher antibody levels are associated with a higher likelihood for disease. Such information improves clinical interpretation. ..
  7. Martin K, Witko Sarsat V. Proteinase 3: the odd one out that became an autoantigen. J Leukoc Biol. 2017;102:689-698 pubmed publisher
    ..Furthermore, these unique structural and functional characteristics of PR3 might be key contributors to the systemic inflammation and to the immune dysregulation observed in GPA. ..
  8. Holle J, Csernok E, Fredenhagen G, Backes M, Bremer J, Gross W. Clinical evaluation of hsPR3-ANCA ELISA for detection of antineutrophil cytoplasmatic antibodies directed against proteinase 3. Ann Rheum Dis. 2010;69:468-9 pubmed publisher
  9. Parulek J, Turkay C, Reuter N, Viola I. Visual cavity analysis in molecular simulations. BMC Bioinformatics. 2013;14 Suppl 19:S4 pubmed publisher
    ..We apply our method to the analysis of MD simulations of Proteinase 3, where we verify the previously described cavities and suggest a new potential cavity to be studied. ..

More Information


  1. Sinden N, Baker M, Smith D, Kreft J, Dafforn T, Stockley R. α-1-antitrypsin variants and the proteinase/antiproteinase imbalance in chronic obstructive pulmonary disease. Am J Physiol Lung Cell Mol Physiol. 2015;308:L179-90 pubmed
  2. Krasselt M, Pierer M, Amann K, Bachmann A, Lindner T. [Clinical Course of an Early Childhood-Onset Granulomatosis with Polyangiitis]. Dtsch Med Wochenschr. 2017;142:904-908 pubmed publisher
  3. Land J, Abdulahad W, Arends S, Sanders J, Stegeman C, Heeringa P, et al. Prospective monitoring of in vitro produced PR3-ANCA does not improve relapse prediction in granulomatosis with polyangiitis. PLoS ONE. 2017;12:e0182549 pubmed publisher
    ..While differences were observed for the serum ANCA titer in relapsing and non-relapsing patients, monitoring in vitro PR3-ANCA IgG production does not improve relapse prediction in GPA patients. ..
  4. Pearce F, Craven A, Merkel P, Luqmani R, Watts R. Global ethnic and geographic differences in the clinical presentations of anti-neutrophil cytoplasm antibody-associated vasculitis. Rheumatology (Oxford). 2017;56:1962-1969 pubmed publisher
    ..This study confirms the previously observed differential occurrence of MPO-AAV and PR3-AAV between different ethnic groups. ..
  5. de Joode A, Sanders J, Puéchal X, Guillevin L, Hiemstra T, Flossmann O, et al. Long term azathioprine maintenance therapy in ANCA-associated vasculitis: combined results of long-term follow-up data. Rheumatology (Oxford). 2017;56:1894-1901 pubmed publisher
    ..ANCA specificity has more effect on relapse-free survival than duration of maintenance therapy and should be used to tailor therapy individually. ..