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Korkmaz, B.* ; Lesner, A.* ; Letast, S.* ; Mahdi, Y.K.* ; Jourdan, M.L.* ; Dallet-Choisy, S.* ; Marchand-Adam, S.* ; Kellenberger, C.* ; Viaud-Massuard, M.C.* ; Jenne, D. ; Gauthier, F.*

Neutrophil proteinase 3 and dipeptidyl peptidase I (cathepsin C) as pharmacological targets in granulomatosis with polyangiitis (Wegener granulomatosis).

Semin. Immunopathol. 35, 411-421 (2013)

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Open Access Green as soon as Postprint is submitted to ZB.

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Neutrophils are among the first cells implicated in acute inflammation. Leaving the blood circulation, they quickly migrate through the interstitial space of tissues and liberate oxidants and other antimicrobial proteins together with serine proteinases. Neutrophil elastase, cathepsin G, proteinase 3 (PR3), and neutrophil serine protease 4 are four hematopoietic serine proteases activated by dipeptidyl peptidase I during neutrophil maturation and are mainly stored in cytoplasmic azurophilic granules. They regulate inflammatory and immune responses after their release from activated neutrophils at inflammatory sites. Membrane-bound PR3 (mbPR3) at the neutrophil surface is the prime antigenic target of antineutrophil cytoplasmic autoantibodies (ANCA) in granulomatosis with polyangiitis (GPA), a vasculitis of small blood vessels and granulomatous inflammation of the upper and/or lower respiratory tracts. The interaction of ANCA with mbPR3 results in excessive activation of neutrophils to produce reactive oxygen species and liberation of granular proteinases to the pericellular environment. In this review, we focus on PR3 and dipeptidyl peptidase I as attractive pharmacological targets whose inhibition is expected to attenuate autoimmune activation of neutrophils in GPA.

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Publication type Article: Journal article

Document type Review

Corresponding Author

Keywords Proteinase3; Dipeptidyl peptidase I (cathepsin C); Neutrophil; Granulomatosis with polyangiitis; ANCA; Antineutrophil Cytoplasmic Antibodies ; Human Polymorphonuclear Neutrophils ; Positive Systemic Vasculitis ; Antiproteinase 3 Antibodies ; Papillon-lefevre-syndrome ; Fc-gamma-riiib ; Serine Proteases ; Membrane Proteinase-3 ; Antigen Proteinase-3 ; Catalytic-activity

ISSN (print) / ISBN 1863-2297

Journal Seminars in Immunopathology

Quellenangaben Volume: 35, Issue: 4, Pages: 411-421

Publisher Springer

Publishing Place Berlin ; Heidelberg

Non-patent literature Publications

Institute(s) Lung Health and Immunity (LHI)