MilliporeSigma™ Proteinase 3/PR3, Mouse, Unlabeled, Clone: MCPR3-2,

Catalog No.	Quantity	Conjugate
MABT340MI	100 μg	Unconjugated

Catalog No. MABT340MI Supplier MilliporeSigma™ Supplier No. MABT340

Description

Mouse Monoclonal Antibody

Myeloblastin (EC 3.4.21.76; UniProt P24158; also known as AGP7, C-ANCA antigen, Leukocyte proteinase 3, Neutrophil proteinase 4, NP-4, P29, PR-3, PR3, Wegener autoantigen) is encoded by the PRTN3 (also known as CANCA, MBN, NP4) gene (Gene ID 5657) in human. Proteinase 3 (PR3) is one of four neutral serine proteases (elastase, cathepsin G, PR3, and neutrophil serine protease 4) stored as fully processed mature enzymes in azurophil granules of human neutrophils. Instead of being targeted to granules after their synthesis, some PR3 molecules end up on the surface of neutrophil plasma membrane in either pro- or mature form. The degree of such surface expression is genetically determined, but the surface exposure and pericellular activity of PR3 around neutrophils can be further upregulated upon neutrophil priming and activation. PR3 autoantibody (anti-neutrophil cytoplasmic antibodies or ANCA) is the main pathogenic feature in patients suffering from granulomatosis with polyangiitis (GPA; formerly called Wegener granulomatosis). ANCAs are shown to activate cytokine-primed neutrophils in vitro by cross-linking surface-exposed PR3 and Fcγ receptors. PR3 activity and/or its inactivation by alpha 1-antitrypsin/alpha-1 proteinase inhibitor (α1PI) varies in the human population and contributes to the risk for GPA manifestations either at onset, during relapses, or during systemic progression. PR3 is initially produced with a signal peptide sequence (a.a. 1-25), an N-terminal and a C-terminal propeptide sequence (a.a. 26-27 and 249-256, respectively), the removal of which yields the mature enzyme (a.a. 28-248).

Specifications

• Antigen: Proteinase 3/PR3
• Applications: ELISA, Flow Cytometry, Western Blot
• Classification: Monoclonal
• Clone: MCPR3-2
• Concentration: Please refer to lot specific datasheet.
• Conjugate: Unconjugated
• Formulation: Purified mouse monoclonal IgG1κ antibody in buffer containing 0.1M Tris-Glycine (pH 7.4), 150mM NaCl with 0.05% Sodium Azide.
• Gene: PRTN3, CANCA, MBN, NP4
• Gene Accession No.: P24158
• Host Species: Mouse
• Immunogen: Granule extract from HMC-1/PR3 mast cells expressing transfected human neutrophil PR3 (Sun, J., et al. (1998). J. Immunol. Methods. 211(1-2):111-123).
• Purification Method: Protein G Purified
• Quantity: 100 μg
• Regulatory Status: RUO
• Research Discipline: Cell Structure
• Primary or Secondary: Primary
• Gene ID (Entrez): NP_002768
• Test Specificity: Clone MCPR3-2 detected wild-type PR3 and the catalytically inactive S176A PR3 mutant, but not neutrophil elastase or cathepsin G. Clone MCPR3-2 did not interfere with PR3 enzymatic activity or its interaction with α1-protease inhibitor/α1-PI (Sun, J., et al. (1998). J. Immunol. Methods. 211(1-2):111-123). The ratios of OD readings obtained with PR3-complexed clone MCPR3-2 to the corresponding ODs obtained with PR3-complexed clone MCPR3-3 (Mfr. No. MABF973) in ELISA-based serum PR3 autoantibodies (ANCAs) measurements correlate well with the neutralizing activity of ANCAs in patients serum samples (Silva, F., et al. (2010). J. Autoimmun. 35(4) 299-308).
• Target Species: Human
• Content And Storage: Stable for 1 year at 2°-8°C from date of receipt.
• Form: Purified
• Isotype: IgG1 κ

For Research Use Only. Not for use in diagnostic procedures.