P9099-02H

Proteinase 3 (Proteinase-3, PR3, PR-3, PRTN3, ACPA, Azurophil Granule Protein 7, AGP7, cANCA, c-ANCA Antigen, EC 3.4.21.76, Leukocyte Proteinase 3, MBT, Myeloblastin, MBN, Neutrophil Proteinase 4, NP4, NP-4, p29, Wegener Autoantigen)

Size

£876.00

SKU:: P9099-02H
Purity:: Purified by Protein G affinity chromatography.
Storage Conditions:: -20°C
Supplier:: United States Biological
Host:: Mouse
Immunogen:: Crude granule extract
Clone:: PR3-G2
Format:: Supplied as a liquid in PBS, 0.1% BSA, 0.02% sodium azide.
Extra Details:: Proteinase 3 (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. Applications: Suitable for use in ELISA, Immunohistochemistry, Flow Cytometry and Western Blot. Other applications not tested. Recommended Dilution: Immunohistochemistry (Frozen): 1:50 Flow Cytometry: 1:50 Western Blot: 1:50 Optimal dilutions to be determined by the researcher. Storage and Stability: May be stored at 4°C for short-term only. Aliquot to avoid repeated freezing and thawing. Store at -20°C. 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.

Shipping Conditions:: Blue Ice