Rabbit anti-Human Proteinase 3/PR3 Polyclonal Antibody

Uniprot Id

P24158

Target Species

Human

Target Name

PRTN3

Target Full Name

Myeloblastin

Target Function

Serine protease that degrades elastin, fibronectin, laminin, vitronectin, and collagen types I, III, and IV (in vitro). By cleaving and activating receptor F2RL1/PAR-2, enhances endothelial cell barrier function and thus vascular integrity during neutrophil transendothelial migration. May play a role in neutrophil transendothelial migration, probably when associated with CD177.

Target Involvement

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 cytoplasmatic autoantibodies detected in patient sera (PubMed:2377228, PubMed:2679910). PRTN3 causes emphysema when administered by tracheal insufflation to hamsters (PubMed:3198760).

Target Subcellular Location

Cytoplasmic granule. Secreted. Cell membrane; Peripheral membrane protein; Extracellular side. Membrane raft; Peripheral membrane protein; Extracellular side.

Target Protein Families

Peptidase S1 family, Elastase subfamily

Target Tissue Specificity

Expressed in polymorphonuclear leukocytes (at protein level). Expressed in neutrophils (at protein level). Expressed in differentiating neutrophils.

Target Research Area

Signal Transduction

Target Synonyms

ACPA; AGP 7; AGP7; AGP7 serine proteinase; Azurophil Granule Protein 7; C ANCA; C ANCA antigen; C-ANCA antigen; CANCA; EC 3.4.21.76; Leukocyte proteinase 3; MBN; MBT; MBT WEGENER AUTOANTIGEN; Myeloblastin; Neutrophil proteinase 4; NP 4 ; NP-4; NP4; P29; PR 3; PR-3; PR3; Proteinase 3; Proteinase3; PRTN 3; Prtn3; PRTN3_HUMAN; Serine proteinase neutrophil Wegener granulomatosis autoantigen; Serine proteinase; neutrophil; Wegener autoantigen; Wegener granulomatosis autoantigen

Target Background

Enables enzyme binding activity; serine-type endopeptidase activity; and signaling receptor binding activity. Involved in several processes, including mature conventional dendritic cell differentiation; membrane protein ectodomain proteolysis; and neutrophil extravasation. Located in azurophil granule lumen; cytosol; and plasma membrane raft. Colocalizes with plasma membrane.