Characterization of the CD177 interaction with the ANCA antigen proteinase 3.

ABSTRACT: Proteinase 3 is a serine protease found in neutrophil granules and on the extracellular neutrophil membrane (mPR3). mPR3 is a major antigen for anti-neutrophil cytoplasmic antibodies (PR3-ANCAs), autoantibodies causing fatal autoimmune diseases. In most individuals, a subpopulation of neutrophils also produce CD177, proposed to present additional PR3 on the surface, resulting in CD177^neg/mPR3^low and CD177^pos/mPR3^high neutrophil subsets. A positive correlation has been shown between mPR3 abundance, disease incidence, and clinical outcome. We present here a detailed investigation of the PR3:CD177 complex, verifying the interaction, demonstrating the effect of binding on PR3 proteolytic activity and explaining the accessibility of major PR3-ANCA epi

SUBMITTER: Jerke U 

PROVIDER: S-EPMC5327412 | biostudies-literature | 2017 Feb

REPOSITORIES: biostudies-literature