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 Fcg receptors.
PR3 activity and/or its inactivation by alpha 1-antitrypsin/alpha-1 proteinase inhibitor (a1PI) 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).
Applications:Suitable for use in Affinity Binding Assays, ELISA, Flow Cytometry and Western Blot.
Other applications not tested.
Recommended Dilution:Western Blot: 2ug/ml detected 5ug of purified human neutrophil PR3 under both non-reducing and reducing conditions Optimal dilutions to be determined by the researcher.
Storage and Stability:May be stored at 4℃ for short-term only.
Aliquot to avoid repeated freezing and thawing.
Store at -20℃.
Aliquots are stable for 12 months after receipt.
For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap.
仕様
Size：100ug
抗体種類：Mab
宿主：mouse
Source Antibody：human
Grade：Affinity Purified
Purity：Purified by Protein G affinity chromatography.
Form：Supplied as a liquid in 0.1M Tris-Glycine, pH 7.4, 150mM sodium chloride, 0.05% sodium azide.
Specificity：Recognizes wild-type human PR3 and the catalytically inactive S176A PR3 mutant, but not neutrophil elastase or cathepsin G. Does not react with mouse
Clone # USB：5G193 (MCPR3-2)
Isotype：IgG1,k
Calc Applications Abbrev：E FC WB
Calc Crossreactivity：Hu
Immunogen：Granule extract from HMC-1/PR3 mast cells expressing transfected human neutrophil PR3. Epitope: “North-East” of the substrate-binding pocket and adjacent to clone MCPR3-3 epitope
