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RESEARCH PRODUCT

The Aspect of Anti-Proteinase 3 Antibodies as AECA

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Publisher Summary The discovery of anti-neutrophil cytoplasmic antibodies (ANCA) has caused a great impact on the diagnostic procedure and clinical observation of systemic necrotizing vasculitides like Wegener's granulomatosis (WG), microscopic polyangiitis, Churg- Strauss syndrome and necrotizing glomerulonephritis. There is persuasive circumstantial evidence for a pathogenic role of ANCA in ANCA-related vasculitides involving both neutrophils and vascular endothelial cells. The major stepthrough in the pathogenesis of ANCA related diseases was the identification of proteinase-3 (PR-3) as the main target antigen of ANCA in Wegener's Granulomatosis. PR-3 has been described as a constituent of the azurophil granules of neutrophils. It is a lysosomal protein identical with myeloblastin and a differentiation factor for myeloid precursor cells. Other functions are cleavage of elastin and other proteins of microorganisms and generation of chemotactic activities by forming alpha 1-proteinase inhibitor complexes.