Abstract
Reactivity of proteinase 3 (PR3) was tested against various amino acid and thioester substrates. The best substrate is Boc-Ala-Ala-Nva-SBzl with a kcat/Km value of 1.0 x 10(6) M-1.s-1. We also studied the effect of C-ANCA on PR3 proteolytic activity towards elastin and inactivation by alpha 1-antitrypsin (alpha 1AT). C-ANCA IgG from 8 patients with active Wegener's granulomatosis were tested and found to inhibit elastin degradation by PR3 and to prevent the inactivation of PR3 by alpha 1AT.
Publication types
-
Research Support, Non-U.S. Gov't
-
Research Support, U.S. Gov't, P.H.S.
MeSH terms
-
Amino Acid Sequence
-
Antibodies, Antineutrophil Cytoplasmic
-
Autoantibodies / pharmacology*
-
Elastin / metabolism
-
Granulomatosis with Polyangiitis / immunology*
-
Humans
-
Immunoglobulin G / pharmacology*
-
Molecular Sequence Data
-
Myeloblastin
-
Peptides / chemistry
-
Peptides / metabolism
-
Serine Endopeptidases / immunology
-
Serine Endopeptidases / metabolism*
-
Substrate Specificity
-
alpha 1-Antitrypsin / pharmacology
Substances
-
Antibodies, Antineutrophil Cytoplasmic
-
Autoantibodies
-
Immunoglobulin G
-
Peptides
-
alpha 1-Antitrypsin
-
Elastin
-
Serine Endopeptidases
-
Myeloblastin