PR3 (PRTN3) Mouse Monoclonal Antibody [Clone ID: PR3-G2]

CAT#: AM26220FC-N

PR3 (PRTN3) mouse monoclonal antibody, clone PR3-G2, FITC

Specifications

Product Data

• Clone Name: PR3-G2
• Applications: ELISA, IHC, WB
• Recommended Dilution: Immunohistochemistry on frozen section: The typical starting working dilution is 1:50.
  Flow cytometry: The typical starting working dilution is 1:50.
  Immunoassay.
  Western blot: The typical starting working dilution is 1:50.
• Reactivities: Human
• Host: Mouse
• Isotype: IgG1
• Clonality: Monoclonal
• Immunogen: A crude granule extract
• Specificity: Monoclonal antibody PR3G-2 reacts with human proteinase 3 (PR3), a 30 kDa protein.
• Formulation: PBS
  Label: FITC
  State: Liquid 0.2 µm filtered Ig fraction
  Stabilizer: 1% bovine serum albumin
  Preservative: 0.02% sodium azide
• Concentration: lot specific
• Purification: Protein G
• Conjugation: FITC
• Storage:

  Store at 2 - 8 °C.
  

• Stability: Shelf life: one year from despatch.

• Gene Name: proteinase 3
• Database Link:
  Entrez Gene 5657 Human
• Background: PR3 is a major antigen recognized by autoantibodies directed against cytoplasmic proteins of neutrophilic granulocytes and monocytes (called anti-neutrophil cytoplasmic autoantibodies (ANCA)). ANCA are able to activate primed neutrophils to produce oxygen radicals and release lytic enzymes, including PR3. Proteinase 3 (PR3) was identified as the target antigen of ANCA in Wegener's granulomatosis (WG). ANCA directed against PR3 (PR3-ANCA) can interfere with the binding of PR3 to its physiological inhibitor alpha1-antitrypsin (alpha1-AT) and with the proteolytic activity of PR3. At the site of inflammation, PR3 can cleave the PR3-ANCA complex between these inhibiting ANCA and PR3 itself, leaving active PR3. Autoantibodies to PR3 are potent activators of the 5-lipoxygenase pathway in primed human neutrophils. Extracellular free arachidonic acid, as present at an inflammatory focus, synergizes with such autoantibodies to evoke full-blown lipid mediator generation, granule secretion and respiratory burst. Proteinase 3 (PR3) is a neutral serine proteinase, which is localized in the azurophilic granules of neutrophils and in granules of monocytes and can be detected in the membrane of secretory vesicles. PR3 degrades a number of extracellular matrix proteins such as elastin and inactivates human C1 inhibitor. Membrane-associated PR3 is also able to activate caspase-3 without triggering apoptosis of neutrophils, which is possibly a neutrophil survival mechanism. In addition, PR3 is involved in myeloid differentiation and is, therefore, also called myeloblastin.
• Synonyms: Myeloblastin, MBN, Leukocyte proteinase 3, Proteinase 3, PRTN3, PR-3, Neutrophil proteinase 4, NP-4, P29, Wegener autoantigen, AGP7