Journal of Biological Chemistry
EnzymologyConsequences of cathepsin C inactivation for membrane exposure of proteinase 3, the target antigen in autoimmune vasculitis
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This work was supported by the “Ministère de l'Enseignement Supérieur et de la Recherche,” the “Région Centre-Val de Loire” (Project BPCO-Lyse). This project has received funding from the European Union's Horizon 2020 research and innovation program under Grant Agreement 668036 (RELENT). The authors declare that they have no conflicts of interest with the contents of this article. Responsibility for the information and views set out in this article lies entirely with the authors.
This article contains Figs. S1–S6.
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U. Specks, personal communication.
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S. Seren, S. Dallet-Choisy, F. Gauthier, S. Marchand-Adam, and B. Korkmaz, unpublished results.
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These authors contributed equally to this work.
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The abbreviations used are:
- GPA
granulomatosis with polyangiitis
- α1PI
α-1-proteinase inhibitor
- Ab
antibody
- ABZ
ortho-aminobenzoic acid
- ANCA
anti-neutrophil cytoplasmic autoantibodies
- Bt
biotin
- CatC
CatL, and CatS, cathepsin C, L, and S, respectively
- CG
cathepsin G
- ECL
enhanced chemiluminescence
- EDDnp
N-(2,4-dinitrophenyl)ethylenediamine
- HNE
human neutrophil elastase
- HSC
hematopoietic stem cell(s)
- NSP
neutrophil serine protease
- PLS
Papillon–Lefèvre syndrome
- PR3
proteinase 3
- PR3m
membrane-bound PR3
- WBC
white blood cell(s)
- NET
neutrophil extracellular trap
- IcatC
CatC inhibitor
- PE
phycoerythrin
- APC
allophycocyanin.