{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,5]],"date-time":"2026-10-05T17:24:55Z","timestamp":1791221095611,"version":"4.1.0"},"reference-count":28,"publisher":"Oxford University Press (OUP)","issue":"3","license":[{"start":{"date-parts":[[2002,8,28]],"date-time":"2002-08-28T00:00:00Z","timestamp":1030492800000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/academic.oup.com\/journals\/pages\/open_access\/funder_policies\/chorus\/standard_publication_model"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2002,8,28]]},"abstract":"<jats:title>SUMMARY<\/jats:title>\n               <jats:p>Anti-neutrophil cytoplasmic antibodies (ANCA) against proteinase 3 (PR3) are strongly associated with Wegener's granulomatosis (WG) and are thought to be involved in its pathogenesis. Levels of PR3-ANCA do not always correspond to clinical disease activity.<\/jats:p>\n               <jats:p>To investigate the relationship between functional effects of PR3-ANCA and disease activity, we tested the effect of IgG samples from sera of 43 WG patients, taken during active disease or remission, for their capacity to interfere with the proteolytic activity of PR3. Furthermore, longitudinal sera of seven WG patients were included. The enzymatic activity of PR3 was determined (1) with casein or with a small synthetic substrate and (2) by complexation of PR3 with \u03b11-antitrypsin (\u03b11-AT).<\/jats:p>\n               <jats:p>With a fixed concentration (100\u2003\u03bcg\/ml) of IgG, PR3-ANCA from patients during an active phase of WG had a higher inhibitory capacity towards the proteolytic activity of PR3 and complexation of PR3 with \u03b11-AT than did PR3-ANCA from WG patients during remission. However, the number of PR3-ANCA units that gave 50% inhibition of the PR3 enzymatic activity and its complexation with \u03b11-AT was lower for patients during remission than for patients during an active phase of WG, indicating a stronger inhibitory capacity at a molar base.<\/jats:p>\n               <jats:p>In conclusion, PR3-ANCA from patients during remission had a relatively higher inhibitory capacity towards the enzymatic activity of PR3 than PR3-ANCA from patients during an active phase. This may indicate that during active disease the ANCA titre is increased, but the number of active ANCA molecules that recognize the enzyme-inhibiting epitopes is not increased.<\/jats:p>","DOI":"10.1046\/j.1365-2249.2002.01926.x","type":"journal-article","created":{"date-parts":[[2003,3,11]],"date-time":"2003-03-11T10:33:46Z","timestamp":1047378826000},"page":"562-570","source":"Crossref","is-referenced-by-count":30,"title":["Interference of PR3-ANCA with the enzymatic activity of PR3: differences in patients during active disease or remission of Wegener's granulomatosis"],"prefix":"10.1093","volume":"129","author":[{"given":"Y M","family":"VAN DER GELD","sequence":"first","affiliation":[{"name":"Department of Internal Medicine, University Hospital Groningen"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"A T J","family":"TOOL","sequence":"additional","affiliation":[{"name":"Sanquin Research at CLB and Landsteiner Laboratory Academic Medical Centre, University of Amsterdam, Amsterdam, the Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"J","family":"VIDELER","sequence":"additional","affiliation":[{"name":"Sanquin Research at CLB and Landsteiner Laboratory Academic Medical Centre, University of Amsterdam, Amsterdam, the Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"M","family":"DE HAAS","sequence":"additional","affiliation":[{"name":"Sanquin Research at CLB and Landsteiner Laboratory Academic Medical Centre, University of Amsterdam, Amsterdam, the Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"J W","family":"COHEN TERVAERT","sequence":"additional","affiliation":[{"name":"Department of Internal Medicine, University Hospital Groningen"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"C A","family":"STEGEMAN","sequence":"additional","affiliation":[{"name":"Department of Internal Medicine, University Hospital Groningen"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"P C","family":"LIMBURG","sequence":"additional","affiliation":[{"name":"Department of Internal Medicine, University Hospital Groningen"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"C G M","family":"KALLENBERG","sequence":"additional","affiliation":[{"name":"Department of Internal Medicine, University Hospital Groningen"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"D","family":"ROOS","sequence":"additional","affiliation":[{"name":"Sanquin Research at CLB and Landsteiner Laboratory Academic Medical Centre, University of Amsterdam, Amsterdam, the Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"286","published-online":{"date-parts":[[2002,8,28]]},"reference":[{"key":"2022011221492275000_b1","doi-asserted-by":"crossref","first-page":"425","DOI":"10.1016\/S0140-6736(85)91147-X","article-title":"Autoantibodies against neutrophils and monocytes: tool for diagnosis and marker of disease activity in Wegener's granulomatosis","volume":"1","author":"Van Der Woude","year":"1985","journal-title":"Lancet"},{"key":"2022011221492275000_b2","doi-asserted-by":"crossref","first-page":"2461","DOI":"10.1001\/archinte.1989.00390110055012","article-title":"Association between active Wegener's granulomatosis and anticytoplasmic antibodies","volume":"149","author":"Cohen Tervaert","year":"1989","journal-title":"Arch Intern Med"},{"key":"2022011221492275000_b3","doi-asserted-by":"crossref","first-page":"1577","DOI":"10.1172\/JCI114335","article-title":"Wegener's granulomatosis autoantibodies identify a novel diisopropylfluorophosphate-binding protein in the lysosomes of normal human neutrophils","volume":"84","author":"Goldschmeding","year":"1989","journal-title":"J Clin Invest"},{"key":"2022011221492275000_b4","doi-asserted-by":"crossref","first-page":"1888","DOI":"10.1182\/blood.V74.6.1888.1888","article-title":"Wegener's granulomatosis autoantigen is a novel neutrophil serine proteinase","volume":"74","author":"Niles","year":"1989","journal-title":"Blood"},{"key":"2022011221492275000_b5","doi-asserted-by":"crossref","first-page":"357","DOI":"10.1084\/jem.171.1.357","article-title":"Anti-neutrophil cytoplasm antibodies in Wegener's granulomatosis recognize an elastinolytic enzyme","volume":"171","author":"L\u00fcdemann","year":"1990","journal-title":"J Exp Med"},{"key":"2022011221492275000_b6","doi-asserted-by":"crossref","first-page":"9540","DOI":"10.1016\/S0021-9258(18)92854-1","article-title":"Characterization of proteinase-3 (PR-3), a neutrophil serine proteinase. 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