PR3 Mouse anti-Human, Clone: PR3G-2, Invitrogen
Mouse Monoclonal Antibody
Manufacturer: Invitrogen MA140218
MA1-40218 detects Proteinase 3 from human samples. MA1-40218 has been successfully used in ELISA, flow cytometry, immunohistochemistry (frozen), and Western blot applications. The MA1-40218 immunogen is human PR3. Endotoxin <24 EU/mg.Proteinase 3 (PR3), a 30 kDa protein, 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.
|PBS with 0.1% BSA and no preservative|
|ACPA, AGP7, C-ANCA, CANCA, MBN, MBT, NP4, P29, PR-3, PR3|
|ELISA, Flow Cytometry, Functional Assay, Immunohistochemistry (Frozen), Western Blot|
|Crude alpha granule extract.|
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