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P9099-01A Mouse Anti-Proteinase 3 (PR3)

Specifications
References
Clone Type
Monoclonal
Host
Mouse
Source
Human
Isotype
IgG1
Clone Number
WGM2
Grade
Affinity Purified
Applications
E FC IHC WB
Crossreactivity
Hu
Shipping Temp
Blue Ice
Storage Temp
-20°C

PR3 is a major antigen recognized by autoantibodies directed against cytoplasmic proteins of neutrophilic granulocytes and monocytes (so 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 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 Flow Cytometry, Western Blot, ELISA and Immunohistochemistry. Other applcations not tested.
Recommended Dilutions
Immunohistochemistry (frozen and paraffin): 1:10 Flow Cytometry: 1:10 Western Blot: 1:10 Functional Assays: Blocks the PR3 activity and partially inhibits the binding of human PR3-ANCA to PR3. 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.
Immunogen
Neutrophils (degranulated supernatant)
Form
Supplied as a liquid in PBS, 0.1% BSA.
Purity
Purified by Protein G affinity chromatography.
Specificity
Recognizes human proteinase 3 (PR3).
References
1. Csernok, E et al; Ultrastructural localization of proteinase 3, the target antigen of anti-cytoplasmic |antibodies circulating in Wegener's granulomatosis. Am.J.Pathol. 1990, 137: 1113. 2. Grimminger, F et al; Neutrophil activation by anti-proteinase 3 antibodies in Wegener ́s granulomatosis: role of exogenous arachidonic acid and leukotriene B4 generation. J Exp Med 1996, 184: 15673. Geld van der, Y et al; Characterization of monoclonal antibodies to proteinase 3 (PR3) as candidate tools for epitope mapping of human anti-PR3 autoantibodies. Clin Exp Immunol 1999, 118: 487 4. Csernok, E et al; A critical evaluation of commercial immunoassays for antineutrophil cytoplasmic antibodies directed against proteinase 3 and myeloperoxidase in Wegener’s granulomatosis and microscopic polyangiitis. Rheumatology 2002, 41: 1313 5. Braun, M et al; Proteinase 3, the target antigen of anticytoplasmic antibodies circulating in Wegener’s granulomatosis. Immunolocalization in normal and pathologic tissues. Am J Pathol 1991, 139: 831 6. Braun, M et al; Monoclonal antibody WGM1 directed against proteinase 3: an immunohistochemical marker for naphtol ASD chloroacetate. Hematolo Oncol 1996, 14: 83 7. Pederzoli, M et al: Proteinase-3 induces procaspase-3 activation in the absence of apoptosis: potential role of this compartmentalized activation of membrane-associated procaspase-3 in neutrophils. J Immunol 2005, 174: 6381
USBio References
No references available
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