Inhibitory Anti-Peroxidasin Antibodies in Pulmonary-Renal Syndromes.

ABSTRACT:

Background

Goodpasture syndrome (GP) is a pulmonary-renal syndrome characterized by autoantibodies directed against the NC1 domains of collagen IV in the glomerular and alveolar basement membranes. Exposure of the cryptic epitope is thought to occur via disruption of sulfilimine crosslinks in the NC1 domain that are formed by peroxidasin-dependent production of hypobromous acid. Peroxidasin, a heme peroxidase, has significant structural overlap with myeloperoxidase (MPO), and MPO-ANCA is present both before and at GP diagnosis in some patients. We determined whether autoantibodies directed against peroxidasin are also detected in GP.

Methods

We used ELISA and competitive binding assays to assess the presence and specificity of autoantibodies in serum from patients wit

SUBMITTER: McCall AS 

PROVIDER: S-EPMC6218858 | biostudies-literature | 2018 Nov

REPOSITORIES: biostudies-literature