Effects of carboxy-terminal modifications of proteinase 3 (PR3) on the recognition by PR3-ANCA

Kidney Int. 2003 Feb;63(2):756-60. doi: 10.1046/j.1523-1755.2003.00765.x.

Abstract

Background: Autoantibodies directed against neutrophil proteinase 3 (PR3-ANCA) from patients with Wegener's granulomatosis and microscopic polyangiitis recognize conformational epitopes of PR3. During maturation of neutrophils, PR3 undergoes amino-terminal and carboxy-terminal processing. In contrast to amino-terminal processing, the effects of carboxy-terminal processing on recognition of PR3 by PR3-ANCA remain unknown. Carboxy-terminally modified or tagged recombinant PR3 (rPR3) molecules may be useful for the refinement of diagnostic assays and for the study of biological processes.

Methods: This study was designed to determine whether 293 cells can be used to express specifically designed carboxy-terminal variants of rPR3, and to evaluate the effects of different carboxy-terminal modifications on the recognition by PR3-ANCA in the capture ELISA.

Results: The rPR3-variants secreted into the media supernatants of transfected 293 cells escaped proteolytic processing. Furthermore, in contrast to the effects of amino-terminal pro-peptide deletion on PR3-ANCA binding, carboxy-terminal modifications (deletion and additions) did not significantly affect recognition by PR3-ANCA.

Conclusions: This expression system is ideally suited for the expression of custom-designed carboxy-terminal rPR3 variants, and major conformational effects of carboxy-terminal modifications seem unlikely.

Publication types

  • Research Support, Non-U.S. Gov't
  • Research Support, U.S. Gov't, P.H.S.

MeSH terms

  • Amino Acid Sequence / genetics
  • Antibodies, Antineutrophil Cytoplasmic / immunology*
  • Base Sequence / genetics
  • Cell Line
  • Enzyme-Linked Immunosorbent Assay / methods
  • Genetic Variation
  • Humans
  • Molecular Sequence Data
  • Molecular Structure
  • Myeloblastin
  • Recombinant Proteins / genetics
  • Recombinant Proteins / immunology
  • Serine Endopeptidases / chemistry*
  • Serine Endopeptidases / immunology*

Substances

  • Antibodies, Antineutrophil Cytoplasmic
  • Recombinant Proteins
  • Serine Endopeptidases
  • Myeloblastin