|
|
 Previous Article | Table of Contents | Next Article 
Wegener's granulomatosis autoantigen is a novel neutrophil serine
proteinase [see comments]
JL Niles, RT McCluskey, MF Ahmad and MA Arnaout
Department of Medicine, Massachusetts General Hospital, Charlestown 02129.
Circulating IgG autoantibodies that produce cytoplasmic immunofluorescence
staining of ethanol-fixed normal neutrophils have recently been found in a
large percentage of patients with active Wegener's granulomatosis. Such
autoantibodies are rarely found in other diseases and are therefore
virtually diagnostic of Wegener's granulomatosis. The nature of the
neutrophil antigen defined by these autoantibodies is controversial and the
roles of the antigen and/or autoantibodies in the pathogenesis of Wegener's
granulomatosis are unknown. We studied serum samples that produce the
cytoplasmic pattern of staining from 10 patients with a diagnosis of
Wegener's granulomatosis. By Western blot analysis, all 10 sera reacted
with a 29- Kd neutrophil protein (p29). We generated a mouse monoclonal
antibody directed against this antigen. The monoclonal antibody produced
the same immunofluorescence staining pattern as the serum autoantibodies
and was used to affinity-purify p29. The purified antigen had a novel N-
terminal sequence homologous to members of the serine proteinase family and
bound to radiolabeled diisopropyl fluorophosphate (DFP). We conclude that
the neutrophil antigen responsible for the cytoplasmic staining pattern
produced by autoantibodies in patients with active Wegener's granulomatosis
is a distinctive serine proteinase.
Volume 74,
Issue 6,
pp. 1888-1893,
11/01/1989
Copyright © 1989 by The American Society of Hematology
 CiteULike  Connotea  Del.icio.us  Digg  Reddit  Technorati What's this?
This article has been cited by other articles:
|
|
|
|
 
A. P van Rossum, M. G Huitema, C. A Stegeman, M. Bijl, K. de Leeuw, M. A Van Leeuwen, P. C Limburg, and C. G M Kallenberg
Standardised assessment of membrane proteinase 3 expression. Analysis in ANCA-associated vasculitis and controls
Ann Rheum Dis,
October 1, 2007;
66(10):
1350 - 1355.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
S. Bauer, M. Abdgawad, L. Gunnarsson, M. Segelmark, H. Tapper, and T. Hellmark
Proteinase 3 and CD177 are expressed on the plasma membrane of the same subset of neutrophils
J. Leukoc. Biol.,
February 1, 2007;
81(2):
458 - 464.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
J. C. Jennette, H. Xiao, and R. J. Falk
Pathogenesis of Vascular Inflammation by Anti-Neutrophil Cytoplasmic Antibodies
J. Am. Soc. Nephrol.,
May 1, 2006;
17(5):
1235 - 1242.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
A. Schreiber, B. Otto, X. Ju, M. Zenke, U. Goebel, F. C. Luft, and R. Kettritz
Membrane Proteinase 3 Expression in Patients with Wegener's Granulomatosis and in Human Hematopoietic Stem Cell-Derived Neutrophils
J. Am. Soc. Nephrol.,
July 1, 2005;
16(7):
2216 - 2224.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
M J Gallagher, K G-J Ooi, M Thomas, and M Gavin
ANCA associated pauci-immune retinal vasculitis
Br. J. Ophthalmol.,
May 1, 2005;
89(5):
608 - 611.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
A. P. van Rossum, A. A. Rarok, M. G. Huitema, G. Fassina, P. C. Limburg, and C. G. M. Kallenberg
Constitutive membrane expression of proteinase 3 (PR3) and neutrophil activation by anti-PR3 antibodies
J. Leukoc. Biol.,
December 1, 2004;
76(6):
1162 - 1170.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
B. Korkmaz, S. Attucci, T. Moreau, E. Godat, L. Juliano, and F. Gauthier
Design and Use of Highly Specific Substrates of Neutrophil Elastase and Proteinase 3
Am. J. Respir. Cell Mol. Biol.,
June 1, 2004;
30(6):
801 - 807.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
A. David, Y. Kacher, U. Specks, and I. Aviram
Interaction of proteinase 3 with CD11b/CD18 ({beta}2integrin) on the cell membrane of human neutrophils
J. Leukoc. Biol.,
October 1, 2003;
74(4):
551 - 557.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
B. Hellmich, E. Csernok, and W. L. Gross
20 years with ANCA (antineutrophil cytoplasmic autoantibodies): from seromarker to a major pathogenic player in vasculitis
J. Leukoc. Biol.,
July 1, 2003;
74(1):
1 - 2.
[Full Text]
[PDF]
|
|
|
|
|
|
|
A. A. Rarok, P. C. Limburg, and C. G. M. Kallenberg
Neutrophil-activating potential of antineutrophil cytoplasm autoantibodies
J. Leukoc. Biol.,
July 1, 2003;
74(1):
3 - 15.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
M Griffith and S Brett
The pulmonary physician in critical care * Illustrative case 3: Pulmonary vasculitis
Thorax,
June 1, 2003;
58(6):
543 - 546.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
A. Schreiber, A. Busjahn, F. C. Luft, and R. Kettritz
Membrane Expression of Proteinase 3 Is Genetically Determined
J. Am. Soc. Nephrol.,
January 1, 2003;
14(1):
68 - 75.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
G. A. Preston, C. S. Zarella, W. F. Pendergraft III, E. H. Rudolph, J. J. Yang, S. B. Sekura, J. C. Jennette, and R. J. Falk
Novel Effects of Neutrophil-Derived Proteinase 3 and Elastase on the Vascular Endothelium Involve In Vivo Cleavage of NF-{kappa}B and Proapoptotic Changes in JNK, ERK, and p38 MAPK Signaling Pathways
J. Am. Soc. Nephrol.,
December 1, 2002;
13(12):
2840 - 2849.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
W Pollock, K Clarke, K Gallagher, J Hall, E Luckhurst, R McEvoy, J Melny, J Neil, A Nikoloutsopoulos, T Thompson, et al.
Immunofluorescent patterns produced by antineutrophil cytoplasmic antibodies (ANCA) vary depending on neutrophil substrate and conjugate
J. Clin. Pathol.,
September 1, 2002;
55(9):
680 - 683.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
A. A. Rarok, C. A. Stegeman, P. C. Limburg, and C. G. M. Kallenberg
Neutrophil Membrane Expression of Proteinase 3 (PR3) Is Related to Relapse in PR3-ANCA-Associated Vasculitis
J. Am. Soc. Nephrol.,
September 1, 2002;
13(9):
2232 - 2238.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
Y. M. van der Geld, P. C. Limburg, and C. G. M. Kallenberg
Proteinase 3, Wegener's autoantigen: from gene to antigen
J. Leukoc. Biol.,
February 1, 2001;
69(2):
177 - 190.
[Abstract]
[Full Text]
|
|
|
|
|
|
|
T. Girard, A. Mahr, L.-H. Noel, J.-F. Cordier, P. Lesavre, M.-H. Andre, and L. Guillevin
Are antineutrophil cytoplasmic antibodies a marker predictive of relapse in Wegener's granulomatosis? A prospective study
Rheumatology,
February 1, 2001;
40(2):
147 - 151.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
R. NOWACK, K. SCHWALBE, L.-F. FLORES-SUÁREZ, B. YARD, and F. J. VAN DER WOUDE
Upregulation of CD14 and CD18 on Monocytes In Vitro by Antineutrophil Cytoplasmic Autoantibodies
J. Am. Soc. Nephrol.,
September 1, 2000;
11(9):
1639 - 1646.
[Abstract]
[Full Text]
|
|
|
|
|
|
|
D. Vassilopoulos and G. S. Hoffman
Clinical Utility of Testing for Antineutrophil Cytoplasmic Antibodies
Clin. Vaccine Immunol.,
September 1, 1999;
6(5):
645 - 651.
[Full Text]
[PDF]
|
|
|
|
|
|
|
P. FOUCHER, P. HEERINGA, A. H. PETERSEN, M. G. HUITEMA, E. BROUWER, J. W. COHEN TERVAERT, J. PROP, P. CAMUS, J. J. WEENING, and C. G. M. KALLENBERG
Antimyeloperoxidase-associated Lung Disease . An Experimental Model
Am. J. Respir. Crit. Care Med.,
September 1, 1999;
160(3):
987 - 994.
[Abstract]
[Full Text]
|
|
|
|
|
|
|
C. A Langford and G. S Hoffman
Rare diseases bullet 3: Wegener's granulomatosis
Thorax,
July 1, 1999;
54(7):
629 - 637.
[Full Text]
|
|
|
|
|
|
|
B. R. Ludviksson, M. C. Sneller, K. S. Chua, C. Talar-Williams, C. A. Langford, R. O. Ehrhardt, A. S. Fauci, and W. Strober
Active Wegener's Granulomatosis Is Associated with HLA-DR+ CD4+ T Cells Exhibiting an Unbalanced Th1-Type T Cell Cytokine Pattern: Reversal with IL-10
J. Immunol.,
April 1, 1998;
160(7):
3602 - 3609.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
D Blockmans, E Stevens, G Mariën, and H Bobbaers
Clinical spectrum associated with positive ANCA titres in 94 consecutive patients: is there a relation with PR-3 negative c-ANCA and hypergammaglobulinaemia ?
Ann Rheum Dis,
March 1, 1998;
57(3):
141 - 145.
[Abstract]
[Full Text]
|
|
|
|
|
|
|
J. C. Jennette and R. J. Falk
Small-Vessel Vasculitis
N. Engl. J. Med.,
November 20, 1997;
337(21):
1512 - 1523.
[Full Text]
[PDF]
|
|
|
|
|
|
|
H. M. Gilligan, B. Bredy, H. R. Brady, M.-J. Hebert, H. S. Slayter, Y. Xu, J. Rauch, M. A. Shia, J. S. Koh, and J. S. Levine
Antineutrophil Cytoplasmic Autoantibodies Interact with Primary Granule Constituents on the Surface of Apoptotic Neutrophils in the Absence of Neutrophil Priming
J. Exp. Med.,
December 15, 1996;
184(6):
2231 - 2242.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
A. Sturrock, K. F. Franklin, and J. R. Hoidal
Human Proteinase-3 Expression Is Regulated by PU.1 in Conjunction with a Cytidine-rich Element
J. Biol. Chem.,
December 13, 1996;
271(50):
32392 - 32402.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
N. V. Rao, G. V. Rao, B. C. Marshall, and J. R. Hoidal
Biosynthesis and Processing of Proteinase 3 in U937 Cells
J. Biol. Chem.,
February 9, 1996;
271(6):
2972 - 2978.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
J. K. Rao, M. Weinberger, E. Z. Oddone, N. B. Allen, P. Landsman, and J. R. Feussner
The Role of Antineutrophil Cytoplasmic Antibody (c-ANCA) Testing in the Diagnosis of Wegener Granulomatosis: A Literature Review and Meta-analysis
Ann Intern Med,
December 15, 1995;
123(12):
925 - 932.
[Abstract]
[Full Text]
|
|
|
|
|
|
|
S. Robache-Gallea, V.ér. Morand, J. M. Bruneau, B. Schoot, E. Tagat, E. Réalo, S. Chouaib, and S. Roman-Roman
In Vitro Processing of Human Tumor Necrosis Factor-alpha
J. Biol. Chem.,
October 6, 1995;
270(40):
23688 - 23692.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
B. L. Fanburg, J. L. Niles, and E. J. Mark
Case 52-1993- A 17-Year-Old Girl with Massive Hemoptysis and Acute Oliguric Renal Failure
N. Engl. J. Med.,
December 30, 1993;
329(27):
2019 - 2026.
[Full Text]
|
|
|
|
|
|
|
J. L. Seifter, J. L. Niles, and R. T. McCluskey
Case 13-1993- A 74-Year-Old Woman with Rapidly Progressing Renal Failure after an Upper Respiratory Tract Infection
N. Engl. J. Med.,
April 1, 1993;
328(13):
951 - 958.
[Full Text]
|
|
|
|
|
|
|
U. Specks, T. V. Colby, K. D. Olsen, and R. A. DeRemee
Salivary Gland Involvement in Wegener's Granulomatosis
Arch Otolaryngol Head Neck Surg,
February 1, 1991;
117(2):
218 - 223.
[Abstract]
[PDF]
|
|
|
|
|
