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Proteinase 3, Human, mAb WGM2

Catalog #: HM2171

Quantity: 100 µg

Availability: In stock

Description: Proteinase 3, Human, mAb WGM2

Monoclonal antibody WGM2 reacts with human proteinase 3 (PR3), a 30 kDa protein. 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. Monoclonal antibody WGM2 blocks the PR3 activity and partially inhibits the binding of human PR3-ANCA to PR3.

Specifications

Catalog number HM2171
Product type Monoclonal antibodies
Quantity 100 µg
Formulation 1 ml (100 µg/ml) 0.2 µm filtered antibody solution in PBS, containing 0.1% bovine serum albumin.
Application F, FC, FS, IA, IF, IP, P, W
Use For flow cytometry, Western blotting and immunohistology dilutions to be used depend on detection system applied. It is recommended that users test the reagent and determine their own optimal dilutions. The typical starting working dilution is 1:10. For inhibition of biological activity of PR3 dilutions have to be made according to the amounts of proteinase 3 to be inactivated.
Isotype Mouse IgG1
Species Human
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: 1567
3. 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: 1313v 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: Proeinase-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
Storage and stability Product should be stored at 4°C. Under recommended st orage conditions, product is stable for one year.
Precautions For research use only. Not for use in or on humans or animals or for diagnostics. It is the responsibility of the user to comply with all local/state and Federal rules in the use of this product. Hycult Biotech is not responsible for any patent infringements that might result with the use of or derivation of this product.
Disease Autoimmunity