PRTN3, proteinase 3, 5657

N. diseases: 215; N. variants: 3
Source: ALL
Disease Score gda Association Type Type Original DB Sentence supporting the association PMID PMID Year
CUI: C2347126
Disease: Microscopic Polyarteritis
Microscopic Polyarteritis
0.100 Biomarker disease BEFREE No proteinase 3-ANCA was detected in all MPA patients with CPS. 31573728 2020
CUI: C2347126
Disease: Microscopic Polyarteritis
Microscopic Polyarteritis
0.100 Biomarker disease BEFREE This study was conducted retrospectively at Caen University Hospital and included all consecutive granulomatosis with polyangiitis (GPA) and microscopic polyangiitis (MPA) patients with positive proteinase 3-ANCA or myeloperoxidase-ANCA, from January 2000 or September 2011, respectively, to June 2016. 30824651 2019
CUI: C2347126
Disease: Microscopic Polyarteritis
Microscopic Polyarteritis
0.100 Biomarker disease BEFREE 276/455 patients were classified as MPO-ANCA positive MPA, 4/455 patients were classified as PR3-ANCA positive MPA, 124/455 were MPO-ANCA positive GPA and 51/455 were PR3-ANCA positive GPA. 29887327 2019
CUI: C2347126
Disease: Microscopic Polyarteritis
Microscopic Polyarteritis
0.100 Biomarker disease BEFREE Minimal to mild CS predicted recovery of renal function at 1 year; clinical diagnosis (granulomatosis with polyangiitis versus MPA) and ANCA specificity (proteinase 3 versus MPO) did not. 30102330 2019
CUI: C2347126
Disease: Microscopic Polyarteritis
Microscopic Polyarteritis
0.100 Biomarker disease BEFREE The small vessel vasculitides granulomatosis with polyangiitis (GPA) and microscopic polyangiitis are associated with autoantibodies to neutrophil cytoplasm antigens (ANCA), principally proteinase-3 (PR3) and myeloperoxidase (MPO). 29447930 2018
CUI: C2347126
Disease: Microscopic Polyarteritis
Microscopic Polyarteritis
0.100 Biomarker disease BEFREE To evaluate circulating cytokine profiles in patients with antineutrophil cytoplasmic antibody-associated vasculitis (AAV), classified by antineutrophil cytoplasmic antibody (ANCA) specificity (proteinase 3 ANCA [PR3-ANCA] versus myeloperoxidase ANCA [MPO-ANCA]) or by clinical diagnosis (granulomatosis with polyangiitis [GPA] versus microscopic polyangiitis [MPA]). 29693324 2018
CUI: C2347126
Disease: Microscopic Polyarteritis
Microscopic Polyarteritis
0.100 GeneticVariation disease BEFREE We reviewed the medical records of 133 patients with microscopic polyangiitis (MPA), granulomatosis with polyangiitis (GPA) and eosinophilic GPA (EGPA), who had either myeloperoxidase (MPO)-ANCA, proteinase 3 (PR3)-ANCA or no ANCA, and who had ever achieved the first remission. 28339364 2017
CUI: C2347126
Disease: Microscopic Polyarteritis
Microscopic Polyarteritis
0.100 Biomarker disease BEFREE We sought to characterize IgM ANCA more clearly by creating a novel enzyme-linked immunosorbent assay (ELISA) for IgM antibodies to proteinase 3 [IgM proteinase 3 (PR3)-ANCA], which we applied to two large, clinically well-characterized trial cohorts of patients with granulomatosis with polyangiitis and microscopic polyangiitis. 28076879 2017
CUI: C2347126
Disease: Microscopic Polyarteritis
Microscopic Polyarteritis
0.100 GeneticVariation disease BEFREE In order to provide an update on the recent advances in the pathogenesis, clinical features and novel treatments of lung involvement in systemic vasculitis, a systematic MedLine search has been performed.Most of the data analyzed have confirmed that lung involvement seems to develop more frequently in patients with myeloperoxidase-ANCA-positive AAV, mainly in those with a diagnosis of microscopic polyangiitis (MPA), compared with patients with proteinase 3 ANCA-positive AAV. 27755177 2017
CUI: C2347126
Disease: Microscopic Polyarteritis
Microscopic Polyarteritis
0.100 Biomarker disease BEFREE Among antineutrophil cytoplasmic antibody (ANCA)-associated vasculitides (AAV), granulomatosis with polyangiitis (GPA) and proteinase 3-ANCA-positive AAV (PR3-AAV) are prevalent in European populations, while microscopic polyangiitis (MPA) and myeloperoxidase-ANCA-positive AAV (MPO-AAV) are predominant in the Japanese. 27166610 2016
CUI: C2347126
Disease: Microscopic Polyarteritis
Microscopic Polyarteritis
0.100 GeneticVariation disease BEFREE Moreover, we identified genetic distinctions between granulomatosis with polyangiitis and microscopic polyangiitis and between proteinase 3 ANCA vasculitis and myeloperoxidase ANCA vasculitis. 26443607 2016
CUI: C2347126
Disease: Microscopic Polyarteritis
Microscopic Polyarteritis
0.100 Biomarker disease BEFREE ANCA directed against proteinase 3 (PR3) are preferentially associated with GPA, and anti-myeloperoxidase (MPO) ANCA are associated mainly with MPA and eosinophilic GPA (formerly known as Churg-Strauss syndrome). 24737903 2014
CUI: C2347126
Disease: Microscopic Polyarteritis
Microscopic Polyarteritis
0.100 Biomarker disease BEFREE In addition, these studies have also shown that different AAV subtypes such as granulomatosis with polyangiitis (Wegener's, GPA) and microscopic polyangiitis (MPA) are underpinned by distinct genetic risk factors, with GPA being associated with HLA-DP, SERPINA1 (encoding α1-antitrypsin), PRTN3 (encoding proteinase-3, PR3, the main GPA-related autoantigen) and SEMA6A (semaphorin 6A), whereas MPA has been mainly associated with HLA-DQ. 24854378 2014
CUI: C2347126
Disease: Microscopic Polyarteritis
Microscopic Polyarteritis
0.100 Biomarker disease BEFREE ANCA directed against proteinase 3 (PR3) are preferentially associated with GPA, and anti-myeloperoxidase (MPO) ANCA are associated mainly with MPA and eosinophilic GPA (formerly known as Churg-Strauss syndrome). 23606701 2013
CUI: C2347126
Disease: Microscopic Polyarteritis
Microscopic Polyarteritis
0.100 GeneticVariation disease BEFREE The distinct associations of PR3-ANCA and MPO-ANCA with different HLA class II antigens, which are stronger than those with the associated diseases, suggest a pathogenic role for those ANCAs and indicate that GPA and MPA are different diseases. 23810690 2013
CUI: C2347126
Disease: Microscopic Polyarteritis
Microscopic Polyarteritis
0.100 GeneticVariation disease BEFREE Patients with AAV (n=105) subgrouped as microscopic polyangiitis or granulomatosis with polyangiitis (Wegener's granulomatosis) and myeloperoxidase (MPO) or proteinase 3 (PR3) ANCA positive were compared to a control group of 200 blood donors. 24356554 2013
CUI: C2347126
Disease: Microscopic Polyarteritis
Microscopic Polyarteritis
0.100 Biomarker disease BEFREE This study confirms that the pathogenesis of ANCA-associated vasculitis has a genetic component, shows genetic distinctions between granulomatosis with polyangiitis and microscopic polyangiitis that are associated with ANCA specificity, and suggests that the response against the autoantigen proteinase 3 is a central pathogenic feature of proteinase 3 ANCA-associated vasculitis. 22808956 2012
CUI: C2347126
Disease: Microscopic Polyarteritis
Microscopic Polyarteritis
0.100 Biomarker disease BEFREE Wegener's granulomatosis, microscopic polyangiitis and Churg Strauss syndrome are small-vessel vasculitides associated with anti-neutrophil cytoplasmic antibodies (ANCA) directed against proteinase 3 (PR3) and myeloperoxidase (MPO). 17521322 2007
CUI: C2347126
Disease: Microscopic Polyarteritis
Microscopic Polyarteritis
0.100 Biomarker disease BEFREE Cytoplasmic (c)-ANCA mainly target proteinase 3 (PR3) and are often observed in WG patients, while perinuclear (p)-ANCA predominantly bind to myeloperoxidase (MPO) and are common in patients with MPA and CSS. 15675141 2004
CUI: C2347126
Disease: Microscopic Polyarteritis
Microscopic Polyarteritis
0.100 Biomarker disease BEFREE Antineutrophil cytoplasmic autoantibodies (ANCA) directed to proteinase 3 (PR3-ANCA) or myeloperoxidase (MPO-ANCA) are closely associated with the idiopathic systemic necrotizing vasculitides, in particular Wegener's granulomatosis, microscopic polyangiitis and its renal limited manifestation, and Churg Strauss Syndrome. 12849060 2002