title: Diversity of PR3-ANCA epitope specificity in Wegener's granulomatosis. Analysis using the biosensor technology.
abstract: Wegener's granulomatosis is a systemic disease characterized by the presence of antineutrophil cytoplasm autoantibodies specific for proteinase 3 (PR3-ANCA). The functional characteristics of PR3-ANCA differ between quiescent and active disease, suggesting changes in the properties of the autoantibodies in time. Using biosensor technology, we found that PR3-ANCA of different patients (n = 8) recognize a limited number of overlapping regions on PR3 at the time of diagnosis of Wegener's granulomatosis. This area might cover an immunodominant epitope, common for PR3-ANCA from all patients, irrespective of the size of the total area recognized by an individual autoantibody. Experiments with sera (n = 4) collected at the moment of diagnosis and at the time of relapse showed that the individual epitope specificities of PR3-ANCA change during the course of the disease. These changes in epitope specificity of PR3-ANCA may be responsible for the differences in functional properties of these autoantibodies between various stages of the disease.
authors.affiliation: Department of Internal Medicine, University Hospital Groningen, Groningen, The Netherlands.
authors.forename: Agnieszka A
authors.initials: AA
authors.lastname: Rarok
authors.forename: Ymke M
authors.initials: YM
authors.lastname: van der Geld
authors.forename: Coen A
authors.initials: CA
authors.lastname: Stegeman
authors.forename: Pieter C
authors.initials: PC
authors.lastname: Limburg
authors.forename: Cees G M
authors.initials: CG
authors.lastname: Kallenberg
mesh_terms: D001323:Autoantibodies / Q000276:immunology*; D015374:Biosensing Techniques; D004797:Enzyme-Linked Immunosorbent Assay; D000939:Epitopes / Q000276:immunology / Q000378:metabolism*; D014890:Granulomatosis with Polyangiitis / Q000276:immunology* / Q000473:pathology; D006801:Humans; D053146:Myeloblastin; D009504:Neutrophils / Q000276:immunology* / Q000473:pathology; D012697:Serine Endopeptidases / Q000276:immunology / Q000378:metabolism*
chemical_list: D001323:Autoantibodies; D000939:Epitopes; D012697:Serine Endopeptidases; D053146:Myeloblastin