|
Granulomatosis with polyangiitis
|
0.600 |
Biomarker
|
disease |
BEFREE |
Cells transfected with PR3 showed positive immunofluorescence for ANCA-containing sera from patients with Wegener's granulomatosis.
|
9000544 |
1997 |
|
Granulomatosis with polyangiitis
|
0.600 |
Biomarker
|
disease |
BEFREE |
Characterization of a recombinant proteinase 3, the autoantigen in Wegener's granulomatosis and its reactivity with anti-neutrophil cytoplasmic autoantibodies.
|
8612734 |
1996 |
|
Granulomatosis with polyangiitis
|
0.600 |
Biomarker
|
disease |
BEFREE |
Circulating anti-neutrophilic cytoplasmic autoantibodies targeting proteinase 3 (PR3-ANCA) are a diagnostic and pathogenic hallmark of granulomatosis with polyangiitis (GPA).
|
30054207 |
2018 |
|
Granulomatosis with polyangiitis
|
0.600 |
AlteredExpression
|
disease |
LHGDN |
Constitutive membrane expression of proteinase 3 (PR3) and neutrophil activation by anti-PR3 antibodies.
|
15331626 |
2004 |
|
Granulomatosis with polyangiitis
|
0.600 |
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 |
|
Granulomatosis with polyangiitis
|
0.600 |
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 |
|
Granulomatosis with polyangiitis
|
0.600 |
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 |
|
Granulomatosis with polyangiitis
|
0.600 |
Biomarker
|
disease |
BEFREE |
Eighty-four proteinase 3 (PR3) anti-neutrophil cytoplasmic antibody (ANCA) positive GPA outpatients were prospectively monitored for up to two years and 32 healthy controls were included.
|
28771641 |
2017 |
|
Granulomatosis with polyangiitis
|
0.600 |
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 |
|
Granulomatosis with polyangiitis
|
0.600 |
Biomarker
|
disease |
BEFREE |
Epitopes on proteinase-3 recognized by antibodies from patients with Wegener's granulomatosis.
|
8157982 |
1994 |
|
Granulomatosis with polyangiitis
|
0.600 |
AlteredExpression
|
disease |
BEFREE |
Expression of recombinant proteinase 3, the autoantigen in Wegener's granulomatosis, in insect cells.
|
12191522 |
2002 |
|
Granulomatosis with polyangiitis
|
0.600 |
Biomarker
|
disease |
BEFREE |
Finally, purified IgG fractions from WG patients were examined for their capacity to inhibit binding between PR3 and alpha 1AT.
|
8889441 |
1996 |
|
Granulomatosis with polyangiitis
|
0.600 |
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 |
|
Granulomatosis with polyangiitis
|
0.600 |
Biomarker
|
disease |
BEFREE |
Furthermore, several genetic loci of S. aureus are associated with either PR3-ANCA- or MPO-ANCA-positive AAV, indicating a possible role for pore-forming toxins, such as leukocidins, in PR3-ANCA-positive GPA.
|
28939882 |
2017 |
|
Granulomatosis with polyangiitis
|
0.600 |
Biomarker
|
disease |
BEFREE |
Furthermore, the pathogenesis of ANCA-associated vasculitis has been reviewed with particular emphasis on the role of proteinase 3 (PR3) in fuelling granulomatosis with polyangiitis (GPA) inflammation.
|
31198793 |
2019 |
|
Granulomatosis with polyangiitis
|
0.600 |
Biomarker
|
disease |
BEFREE |
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.
|
28546501 |
2017 |
|
Granulomatosis with polyangiitis
|
0.600 |
Biomarker
|
disease |
BEFREE |
Genes up-regulated in WG PBMCs were involved in myeloid differentiation, and these included the WG autoantigen PR3.
|
20155833 |
2010 |
|
Granulomatosis with polyangiitis
|
0.600 |
Biomarker
|
disease |
BEFREE |
Genetics and pathophysiology of granulomatosis with polyangiitis (GPA) and its main autoantigen proteinase 3.
|
27559009 |
2016 |
|
Granulomatosis with polyangiitis
|
0.600 |
GeneticVariation
|
disease |
BEFREE |
Granuloma formation, as seen in PR3-ANCA-associated GPA, is not well explained by the presence of autoantibodies in experimental models.Here, T cells seem crucial.
|
23810690 |
2013 |
|
Granulomatosis with polyangiitis
|
0.600 |
Biomarker
|
disease |
BEFREE |
His serum proteinase 3-antineutrophil cytoplasmic antibody (PR3-ANCA) level was also elevated to 31.1 U/mL, but granulomatosis with polyangiitis was not observed.
|
28069788 |
2017 |
|
Granulomatosis with polyangiitis
|
0.600 |
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 |
|
Granulomatosis with polyangiitis
|
0.600 |
Biomarker
|
disease |
BEFREE |
In the ICU group, patients predominantly showed granulomatosis with polyangiitis and proteinase-3 ANCAs.
|
28382598 |
2017 |
|
Granulomatosis with polyangiitis
|
0.600 |
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 |
|
Granulomatosis with polyangiitis
|
0.600 |
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 |
|
Granulomatosis with polyangiitis
|
0.600 |
Biomarker
|
disease |
BEFREE |
Intravascular large B-cell lymphoma with a high titer of proteinase-3-anti-neutrophil cytoplasmic antibody mimicking granulomatosis with polyangiitis.
|
27398728 |
2019 |