PRTN3
Function
Serine protease that degrades elastin, fibronectin, laminin, vitronectin, and collagen types I, III, and IV (in vitro) (PubMed:2033050, PubMed:28240246, PubMed:3198760). By cleaving and activating receptor F2RL1/PAR-2, enhances endothelial cell barrier function and thus vascular integrity during neutrophil transendothelial migration (PubMed:23202369). May play a role in neutrophil transendothelial migration, probably when associated with CD177 (PubMed:22266279).
Involvement in disease
Is the major autoantigen in anti-neutrophil cytoplasmic autoantibody (ANCA)-associated vasculitis (Wegener's granulomatosis) (PubMed:2377228, PubMed:2679910). This complex, systemic disease is characterized by granulomatous inflammation with necrotizing lesions in the respiratory tract, glomerulonephritis, vasculitis, and anti-neutrophil cytoplasmic autoantibodies detected in patient sera (PubMed:2377228, PubMed:2679910). PRTN3 causes emphysema when administered by tracheal insufflation to hamsters (PubMed:3198760).
Sequence Similarities
Belongs to the peptidase S1 family. Elastase subfamily.
Tissue Specificity
Expressed in polymorphonuclear leukocytes (at protein level) (PubMed:2033050, PubMed:3198760, PubMed:7897245). Expressed in neutrophils (at protein level) (PubMed:17244676, PubMed:18462208, PubMed:21193407, PubMed:22266279, PubMed:28240246). Expressed in differentiating neutrophils (PubMed:18462208).
Cellular localization
- Cytoplasmic granule
- Secreted
- Cell membrane
- Peripheral membrane protein
- Extracellular side
- Membrane raft
- Peripheral membrane protein
- Extracellular side
- Localizes predominantly to azurophil granules (primary secretory granules) in neutrophils (PubMed:18462208, PubMed:2033050, PubMed:3198760, PubMed:7897245). Secreted upon neutrophil stimulation by TNF-alpha, lipopolysaccharide (LPS), fMLP and CXCL8/IL8 or during neutrophil transmigration (PubMed:22266279, PubMed:28240246). Following secretion tethered to the cell membrane by CD177 (PubMed:18462208, PubMed:22266279).
Alternative names
MBN, PRTN3, Myeloblastin, AGP7, C-ANCA antigen, Leukocyte proteinase 3, Neutrophil proteinase 4, P29, Wegener autoantigen, PR-3, PR3, NP-4