title: Glycosylation of proteinase 3 (PR3) is not required for its reactivity with antineutrophil cytoplasmic antibodies (ANCA) in Wegener's granulomatosis.
abstract: OBJECTIVE
  The glycosylation status of autoantigens appears to be crucial for the pathogenesis of some autoimmune diseases, since carbohydrates play a crucial role in the distinction of self from non-self. Proteinase 3 (PR3), the main target antigen for anti-neutrophil cytoplasmic antibodies (ANCA) in patients with Wegener's granulomatosis (WG), contains two Asn-linked glycosylation sites. The present study explores the influence of the glycosylation status of PR3 on the PR3 recognition by ANCA in a well characterized population of patients with WG.
  
  
  METHODS
  Forty-four patients with WG (459 serum samples) who participated in a multicenter randomized trial, were tested by capture ELISA for ANCA against PR3 and deglycosylated recombinant variants of PR3.
  
  
  RESULTS
  The patients were followed for a median of 27 months, and the median number of serum samples per patient was 10. At baseline, the correlation between the levels of ANCA against PR3 and against all the deglycosylated recombinant variants of PR3 were greater than 0.94 (?<0.001 for all the comparisons). Longitudinal analyses comparing the levels of ANCA against PR3 versus all the deglycosylated recombinant variants of PR3, using linear mixed models, showed no significant statistical differences (rho >or=0.90 in all cases).
  
  
  CONCLUSIONS
  The glycosylation status of PR3 has no impact on its recognition by ANCA in WG.
authors: Thoracic Disease Research Unit, Division of Pulmonary and Critical Care Medicine, Mayo Clinic, Rochester, MN, USA.
authors: J D
authors: JD
authors: Finkielman
authors: P A
authors: PA
authors: Merkel
authors: D
authors: D
authors: Schroeder
authors: G S
authors: GS
authors: Hoffman
authors: R
authors: R
authors: Spiera
authors: E W
authors: EW
authors: St Clair
authors: J C
authors: JC
authors: Davis
authors: W J
authors: WJ
authors: McCune
authors: A
authors: A
authors: Lears
authors: S R
authors: SR
authors: Ytterberg
authors: A M
authors: AM
authors: Hummel
authors: M A
authors: MA
authors: Viss
authors: T
authors: T
authors: Peikert
authors: J H
authors: JH
authors: Stone
authors: U
authors: U
authors: Specks
mesh_terms: D000328:Adult; D019268:Antibodies, Antineutrophil Cytoplasmic / Q000276:immunology* / Q000378:metabolism; D000937:Antigen-Antibody Reactions; D002461:Cell Line, Transformed; D005260:Female; D006031:Glycosylation; D014890:Granulomatosis with Polyangiitis / Q000097:blood / Q000276:immunology*; D006801:Humans; D008297:Male; D008875:Middle Aged; D053146:Myeloblastin / Q000276:immunology* / Q000378:metabolism
chemical_list: D019268:Antibodies, Antineutrophil Cytoplasmic; D053146:Myeloblastin