'Classic' Anti-Neutrophil Cytoplasmic Autoantibodies (cANCA), 'Wegener's Autoantigen' and Their Immunopathogenic Role in Wegener's Granulomatosis
Identifieur interne : 001689 ( Istex/Curation ); précédent : 001688; suivant : 001690'Classic' Anti-Neutrophil Cytoplasmic Autoantibodies (cANCA), 'Wegener's Autoantigen' and Their Immunopathogenic Role in Wegener's Granulomatosis
Auteurs : Wolfgang L. Gross [Allemagne] ; Elena Csernok [Allemagne] ; Brigitte K. Flesch [Allemagne]Source :
- Journal of Autoimmunity [ 0896-8411 ] ; 1993.
Abstract
Abstract: Wegener's autoantigen (WA), a 29 kD multifunctional protein, is the principal target antigen of autoantibodies associated with Wegener's granulomatosis (WG). WA was first identified as proteinase 3 (PR3), which is now known to be identical with myeloblastin and AGP7. Like other lysosomal proteins, WA/PR3 displays enzymatic activity, differentiation factor activity for myeloid precursor cells, and antimicrobial functions. Neutrophilic polymorphonuclear leukocytes (PMN) and a subpopulation of monocytes contain high levels of WA/PR3 in their myeloperoxidase-positive granules. The autoantibodies from WG sera produce a finely granular, centrally accentuated fluorescence pattern on PMN and monocytes and have been designated 'classic' pattern antineutrophil cytoplasmic autoantibodies (cANCA). However, PMN/monocyte activation (in vitro/ex vivo) is associated with the translocation of WA/PR3 on the cytoplasm membrane. WA/PR3 is accessible to the WG-associated autoantibody: cANCA stimulate cytokine-preactivated PMN to produce oxygen radicals and to degranulate. Furthermore, cANCA interfere with the biological functions of WA/PR3 (e.g. inhibition of elastinolytic activity). Hence, cANCA represents not only the best seromarker for WG so far available, but several lines of evidence indicate that the autoantibodies against WA/PR3 play a major role in the pathogenesis of this enigmatic disease.
Url:
DOI: 10.1006/jaut.1993.1015
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<record><TEI wicri:istexFullTextTei="biblStruct"><teiHeader><fileDesc><titleStmt><title xml:lang="en">'Classic' Anti-Neutrophil Cytoplasmic Autoantibodies (cANCA), 'Wegener's Autoantigen' and Their Immunopathogenic Role in Wegener's Granulomatosis</title><author><name sortKey="Gross, Wolfgang L" sort="Gross, Wolfgang L" uniqKey="Gross W" first="Wolfgang L." last="Gross">Wolfgang L. Gross</name><affiliation wicri:level="1"><mods:affiliation>Medical University in Lübeck, Department of Clinical Rheumatology, and Rhemaklinik Bad Bramstedt, Germany</mods:affiliation><country xml:lang="fr">Allemagne</country><wicri:regionArea>Medical University in Lübeck, Department of Clinical Rheumatology, and Rhemaklinik Bad Bramstedt</wicri:regionArea></affiliation></author><author><name sortKey="Csernok, Elena" sort="Csernok, Elena" uniqKey="Csernok E" first="Elena" last="Csernok">Elena Csernok</name><affiliation wicri:level="1"><mods:affiliation>Medical University in Lübeck, Department of Clinical Rheumatology, and Rhemaklinik Bad Bramstedt, Germany</mods:affiliation><country xml:lang="fr">Allemagne</country><wicri:regionArea>Medical University in Lübeck, Department of Clinical Rheumatology, and Rhemaklinik Bad Bramstedt</wicri:regionArea></affiliation></author><author><name sortKey="Flesch, Brigitte K" sort="Flesch, Brigitte K" uniqKey="Flesch B" first="Brigitte K." last="Flesch">Brigitte K. Flesch</name><affiliation wicri:level="1"><mods:affiliation>Medical University in Lübeck, Department of Clinical Rheumatology, and Rhemaklinik Bad Bramstedt, Germany</mods:affiliation><country xml:lang="fr">Allemagne</country><wicri:regionArea>Medical University in Lübeck, Department of Clinical Rheumatology, and Rhemaklinik Bad Bramstedt</wicri:regionArea></affiliation></author></titleStmt><publicationStmt><idno type="wicri:source">ISTEX</idno><idno type="RBID">ISTEX:DD014ECDA9AFA044D59DB02C001A5B7154E116EC</idno><date when="1993" year="1993">1993</date><idno type="doi">10.1006/jaut.1993.1015</idno><idno type="url">https://api.istex.fr/ark:/67375/6H6-X1WXBNS4-N/fulltext.pdf</idno><idno type="wicri:Area/Istex/Corpus">001689</idno><idno type="wicri:explorRef" wicri:stream="Istex" wicri:step="Corpus" wicri:corpus="ISTEX">001689</idno><idno type="wicri:Area/Istex/Curation">001689</idno></publicationStmt><sourceDesc><biblStruct><analytic><title level="a" type="main" xml:lang="en">'Classic' Anti-Neutrophil Cytoplasmic Autoantibodies (cANCA), 'Wegener's Autoantigen' and Their Immunopathogenic Role in Wegener's Granulomatosis</title><author><name sortKey="Gross, Wolfgang L" sort="Gross, Wolfgang L" uniqKey="Gross W" first="Wolfgang L." last="Gross">Wolfgang L. Gross</name><affiliation wicri:level="1"><mods:affiliation>Medical University in Lübeck, Department of Clinical Rheumatology, and Rhemaklinik Bad Bramstedt, Germany</mods:affiliation><country xml:lang="fr">Allemagne</country><wicri:regionArea>Medical University in Lübeck, Department of Clinical Rheumatology, and Rhemaklinik Bad Bramstedt</wicri:regionArea></affiliation></author><author><name sortKey="Csernok, Elena" sort="Csernok, Elena" uniqKey="Csernok E" first="Elena" last="Csernok">Elena Csernok</name><affiliation wicri:level="1"><mods:affiliation>Medical University in Lübeck, Department of Clinical Rheumatology, and Rhemaklinik Bad Bramstedt, Germany</mods:affiliation><country xml:lang="fr">Allemagne</country><wicri:regionArea>Medical University in Lübeck, Department of Clinical Rheumatology, and Rhemaklinik Bad Bramstedt</wicri:regionArea></affiliation></author><author><name sortKey="Flesch, Brigitte K" sort="Flesch, Brigitte K" uniqKey="Flesch B" first="Brigitte K." last="Flesch">Brigitte K. Flesch</name><affiliation wicri:level="1"><mods:affiliation>Medical University in Lübeck, Department of Clinical Rheumatology, and Rhemaklinik Bad Bramstedt, Germany</mods:affiliation><country xml:lang="fr">Allemagne</country><wicri:regionArea>Medical University in Lübeck, Department of Clinical Rheumatology, and Rhemaklinik Bad Bramstedt</wicri:regionArea></affiliation></author></analytic><monogr></monogr><series><title level="j">Journal of Autoimmunity</title><title level="j" type="abbrev">YJAUT</title><idno type="ISSN">0896-8411</idno><imprint><publisher>ELSEVIER</publisher><date type="published" when="1993">1993</date><biblScope unit="volume">6</biblScope><biblScope unit="issue">2</biblScope><biblScope unit="page" from="171">171</biblScope><biblScope unit="page" to="184">184</biblScope></imprint><idno type="ISSN">0896-8411</idno></series></biblStruct></sourceDesc><seriesStmt><idno type="ISSN">0896-8411</idno></seriesStmt></fileDesc><profileDesc><textClass></textClass><langUsage><language ident="en">en</language></langUsage></profileDesc></teiHeader><front><div type="abstract" xml:lang="en">Abstract: Wegener's autoantigen (WA), a 29 kD multifunctional protein, is the principal target antigen of autoantibodies associated with Wegener's granulomatosis (WG). WA was first identified as proteinase 3 (PR3), which is now known to be identical with myeloblastin and AGP7. Like other lysosomal proteins, WA/PR3 displays enzymatic activity, differentiation factor activity for myeloid precursor cells, and antimicrobial functions. Neutrophilic polymorphonuclear leukocytes (PMN) and a subpopulation of monocytes contain high levels of WA/PR3 in their myeloperoxidase-positive granules. The autoantibodies from WG sera produce a finely granular, centrally accentuated fluorescence pattern on PMN and monocytes and have been designated 'classic' pattern antineutrophil cytoplasmic autoantibodies (cANCA). However, PMN/monocyte activation (in vitro/ex vivo) is associated with the translocation of WA/PR3 on the cytoplasm membrane. WA/PR3 is accessible to the WG-associated autoantibody: cANCA stimulate cytokine-preactivated PMN to produce oxygen radicals and to degranulate. Furthermore, cANCA interfere with the biological functions of WA/PR3 (e.g. inhibition of elastinolytic activity). Hence, cANCA represents not only the best seromarker for WG so far available, but several lines of evidence indicate that the autoantibodies against WA/PR3 play a major role in the pathogenesis of this enigmatic disease.</div></front></TEI></record>Pour manipuler ce document sous Unix (Dilib)
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