Pauci-immune Glomerulonephritis associated with Granulomatosis with Polyangiitis
|
0.100 |
Biomarker
|
disease |
BEFREE |
Granulomatosis with polyangiitis (Wegener granulomatosis): A proteinase-3 driven disease?
|
28495524 |
2018 |
Pauci-immune Glomerulonephritis associated with Granulomatosis with Polyangiitis
|
0.100 |
Biomarker
|
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 |
Pauci-immune Glomerulonephritis associated with Granulomatosis with Polyangiitis
|
0.100 |
Biomarker
|
disease |
BEFREE |
Deciphering the molecular associations that PR3 can make with its cognate partners might help to understand its pathophysiological significance in Wegener's granulomatosis and the potential role of ANCA as modulator of PR3 functions.
|
19881350 |
2010 |
Pauci-immune Glomerulonephritis associated with Granulomatosis with Polyangiitis
|
0.100 |
Biomarker
|
disease |
BEFREE |
Proteinase 3 (PR3)-specific antineutrophil cytoplasmic antibodies (ANCA) are highly specific for the autoimmune small vessel vasculitis, Wegener's granulomatosis (WG).
|
20810247 |
2010 |
Pauci-immune Glomerulonephritis associated with Granulomatosis with Polyangiitis
|
0.100 |
Biomarker
|
disease |
BEFREE |
Genes up-regulated in WG PBMCs were involved in myeloid differentiation, and these included the WG autoantigen PR3.
|
20155833 |
2010 |
Pauci-immune Glomerulonephritis associated with Granulomatosis with Polyangiitis
|
0.100 |
Biomarker
|
disease |
BEFREE |
The pathognomonic ANCA in WG is typically directed against proteinase 3 (PR3).
|
18799062 |
2009 |
Pauci-immune Glomerulonephritis associated with Granulomatosis with Polyangiitis
|
0.100 |
Biomarker
|
disease |
BEFREE |
Increased amounts of anti-neutrophil cytoplasm antibody (ANCA) directed against proteinase 3 (PR3) are a diagnostic and pathogenic hallmark of full-blown Wegener's granulomatosis (WG).
|
18826496 |
2008 |
Pauci-immune Glomerulonephritis associated with Granulomatosis with Polyangiitis
|
0.100 |
GeneticVariation
|
disease |
BEFREE |
Proteinase 3 (PR3), the target antigen of antineutrophil cytoplasm autoantibodies, which are found in patients with Wegener granulomatosis, is a neutrophil serine protease localized within cytoplasmic granules.
|
18854317 |
2008 |
Pauci-immune Glomerulonephritis associated with Granulomatosis with Polyangiitis
|
0.100 |
AlteredExpression
|
disease |
BEFREE |
Proteinase 3 (PR3), a serine proteinase contained in neutrophil azurophilic granules, is considered a risk factor for vasculitides and rheumatoid arthritis when expressed on the outer leaflet of neutrophil plasma membrane and is the preferred target of antineutrophil cytoplasm autoantibodies (ANCA) in Wegener granulomatosis.
|
17712045 |
2007 |
Pauci-immune Glomerulonephritis associated with Granulomatosis with Polyangiitis
|
0.100 |
Biomarker
|
disease |
BEFREE |
For testing of whether the HLA system influences membrane PR3 percentage, membrane PR3 typing and HLA typing of 51 unrelated patients with Wegener's granulomatosis and 49 normal control subjects was performed.
|
17005932 |
2006 |
Pauci-immune Glomerulonephritis associated with Granulomatosis with Polyangiitis
|
0.100 |
GeneticVariation
|
disease |
BEFREE |
We examined five additional SNPs and a sixth SNP haplotype within the PRTN3 promoter region in a family-based association analysis and found no evidence that mutations within PRTN3 are associated with WG diagnosis.
|
16026591 |
2005 |
Pauci-immune Glomerulonephritis associated with Granulomatosis with Polyangiitis
|
0.100 |
AlteredExpression
|
disease |
BEFREE |
Elevated levels of PR3 expression have been observed in WG patients and high levels of PR3 expression corresponded to increased risk of disease relapses.
|
15675141 |
2004 |
Pauci-immune Glomerulonephritis associated with Granulomatosis with Polyangiitis
|
0.100 |
AlteredExpression
|
disease |
BEFREE |
These data may be relevant in view of the observed relation between membrane expression of PR3 on nonprimed neutrophils of patients with WG and their susceptibility for relapses.
|
15331626 |
2004 |
Pauci-immune Glomerulonephritis associated with Granulomatosis with Polyangiitis
|
0.100 |
AlteredExpression
|
disease |
BEFREE |
Earlier studies have shown that circulating leucocytes from patients with Wegener's granulomatosis show elevated proteinase 3 surface expression and mRNA levels.
|
15498036 |
2004 |
Pauci-immune Glomerulonephritis associated with Granulomatosis with Polyangiitis
|
0.100 |
AlteredExpression
|
disease |
BEFREE |
Neutrophil mPR3 expression was studied in 35 patients with WG, 15 patients with other inflammatory diseases, 125 healthy volunteers, and 27 (15 monozygotic and 12 dizygotic) pairs of twins.
|
12506139 |
2003 |
Pauci-immune Glomerulonephritis associated with Granulomatosis with Polyangiitis
|
0.100 |
Biomarker
|
disease |
BEFREE |
Proteinase 3, the main autoantigen in Wegener's granulomatosis (WG), can degrade SLPI proteolytically.
|
12519404 |
2003 |
Pauci-immune Glomerulonephritis associated with Granulomatosis with Polyangiitis
|
0.100 |
AlteredExpression
|
disease |
BEFREE |
Interestingly, the parenchymal cells (pneumocytes type I and II) and macrophages, and not the neutrophils, express PR-3 most strongly and may contribute to lung damage in patients with WG via direct interaction with antineutrophil cytoplasmic antobodies (ANCA).
|
12010574 |
2002 |
Pauci-immune Glomerulonephritis associated with Granulomatosis with Polyangiitis
|
0.100 |
AlteredExpression
|
disease |
BEFREE |
Expression of recombinant proteinase 3, the autoantigen in Wegener's granulomatosis, in insect cells.
|
12191522 |
2002 |
Pauci-immune Glomerulonephritis associated with Granulomatosis with Polyangiitis
|
0.100 |
Biomarker
|
disease |
BEFREE |
Wegener's granulomatosis (WG) is a necrotizing vasculitis characterized by clonal expansions of T cells and production of antibodies against proteinase 3.
|
11838849 |
2002 |
Pauci-immune Glomerulonephritis associated with Granulomatosis with Polyangiitis
|
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 |
Pauci-immune Glomerulonephritis associated with Granulomatosis with Polyangiitis
|
0.100 |
Biomarker
|
disease |
BEFREE |
Previous studies have shown that proteinase 3 (PR3), the major autoantigen in Wegener's granulomatosis, specifically binds to endothelial cells and plays a possible role in activation of these cells by enhancing interleukin-8 production, thus providing a chemotactic and activating stimulus for PMN.
|
11316851 |
2001 |
Pauci-immune Glomerulonephritis associated with Granulomatosis with Polyangiitis
|
0.100 |
Biomarker
|
disease |
BEFREE |
This review will focus on PR3 and the characteristics of PR3 that might implicate this particular antigen in the pathogenesis of WG and as target for immunotherapy in myeloid leukemias.
|
11272267 |
2001 |
Pauci-immune Glomerulonephritis associated with Granulomatosis with Polyangiitis
|
0.100 |
Biomarker
|
disease |
BEFREE |
De novo PR3 synthesis was evaluated by metabolic labeling with [35S] methionine followed by immunoprecipitation using anti-neutrophil cytoplasmic antibodies from serum of patients with active Wegener's granulomatosis and mouse monoclonal anti-native PR3 antibodies.
|
11036837 |
2000 |
Pauci-immune Glomerulonephritis associated with Granulomatosis with Polyangiitis
|
0.100 |
GeneticVariation
|
disease |
BEFREE |
This study excludes certain PR3 epitope variants as autoantigenic stimuli in WG, since the Val119Ile polymorphism showed no differences between patients and controls.
|
11115080 |
2000 |
Pauci-immune Glomerulonephritis associated with Granulomatosis with Polyangiitis
|
0.100 |
AlteredExpression
|
disease |
BEFREE |
We assessed the expression of PR-3 in kidney biopsies of 15 patients with WG by immunohistochemistry (IHC) and in situ hybridization (ISH).
|
10844610 |
2000 |