monoclonal antibody to pr3 / c-anca - purified · am26221pu-n: monoclonal antibody to pr3 / c-anca...
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OriGene EU
Acris Antibodies GmbHSchillerstr. 532052 HerfordGERMANYPhone: +49-5221-34606-0Fax: [email protected]
OriGene Technologies Inc.
9620 Medical Center Drive, Ste 200Rockville, MD 20850UNITED STATESPhone: +1-888-267-4436Fax: [email protected]
AM26221PU-N
Monoclonal Antibody to PR3 / C-ANCA - PurifiedAlternate names: AGP7, Leukocyte proteinase 3, MBN, Myeloblastin, NP-4, Neutrophil proteinase 4, P29,
PR-3, PRTN3, Proteinase 3, Wegener autoantigen
Catalog No.: AM26221PU-N
Quantity: 0.1 mg
Concentration: 0.1 mg/ml
Background: PR3 is a major antigen recognized by autoantibodies directed against cytoplasmic proteinsof neutrophilic granulocytes and monocytes (so called anti-neutrophil cytoplasmicautoantibodies (ANCA)). ANCA are able to activate primed neutrophils to produce oxygenradicals and release lytic enzymes, including PR3. Proteinase 3 (PR3) was identified as thetarget antigen of ANCA in Wegener's granulomatosis (WG). ANCA directed against PR3(PR3-ANCA) can interfere with the binding of PR3 to its physiological inhibitoralpha1-antitrypsin (alpha1-AT) and with the proteolytic activity of PR3. At the site ofinflammation PR3 can cleave the complex between these inhibiting ANCA and PR3 itself,leaving active PR3. Autoantibodies to PR3 are potent activators of the 5-lipoxygenasepathway in primed human neutrophils. Extracellular free arachidonic acid, as present at aninflammatory focus, synergizes with such autoantibodies to evoke full-blown lipidmediator generation, granule secretion and respiratory burst. Proteinase 3 (PR3) is aneutral serine proteinase, which is localized in the azurophilic granules of neutrophils andin granules of monocytes and can be detected in the membrane of secretory vesicles. PR3degrades a number of extracellular matrix proteins such as elastin and inactivates humanC1 inhibitor. Membrane-associated PR3 is also able to activate caspase-3 without triggeringapoptosis of neutrophils, which is possibly a neutrophil survival mechanism. In addition,PR3 is involved in myeloid differentiation and is, therefore, also called myeloblastin.
Uniprot ID: P24158
NCBI: NP_002768.3
GeneID: 5657
Host / Isotype: Mouse / IgG1
RecommendedIsotype Controls:
SM10P (for use in human samples), AM03095PU-N
Clone: WGM2
Format: State: Liquid 0.2 µm filtered Ig fractionPurification: Protein G ChromatographyBuffer System: PBSPreservatives: NoneStabilizers: 0.1% BSA
For research and in vitro use only. Not for diagnostic or therapeutic work.Material Safety Datasheets are available at www.acris-antibodies.com or on request.
Acris Antibodies is now part of the OriGene family. Learn more at www.origene.com
MS/20180409
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AM26221PU-N: Monoclonal Antibody to PR3 / C-ANCA - Purified
Applications: Flow Cytometry.Immunoassays.Western blot.The typical starting working dilution is 1/10.Functional assays: Blocks the PR3 activity and partially inhibits the binding of humanPR3-ANCA to PR3. Immunohistochemistry on Frozen and Paraffin Sections.Other applications not tested. Optimal dilutions are dependent on conditions and shouldbe determined by the user.
Specificity: This Monoclonal antibody WGM2 reacts with Human Proteinase 3 (PR3), a 30 kDa protein.
Species Reactivity: Tested: Human
Storage: Store undiluted at 2-8°C.DO NOT FREEZE!Shelf life: one year from despatch.
General Readings: 1. Csernok E, Lüdemann J, Gross WL, Bainton DF. Ultrastructural localization of proteinase3, the target antigen of anti-cytoplasmic antibodies circulating in Wegener'sgranulomatosis. Am J Pathol. 1990 Nov;137(5):1113-20. PubMed PMID: 2240162.2. Grimminger F, Hattar K, Papavassilis C, Temmesfeld B, Csernok E, Gross WL, et al.Neutrophil activation by anti-proteinase 3 antibodies in Wegener's granulomatosis: role ofexogenous arachidonic acid and leukotriene B4 generation. J Exp Med. 1996 Oct1;184(4):1567-72. PubMed PMID: 8879231.3. Van Der Geld YM, Limburg PC, Kallenberg CG. Characterization of monoclonal antibodiesto proteinase 3 (PR3) as candidate tools for epitope mapping of human anti-PR3autoantibodies. Clin Exp Immunol. 1999 Dec;118(3):487-96. PubMed PMID: 10594572.4. Csernok E, Ahlquist D, Ullrich S, Gross WL. A critical evaluation of commercialimmunoassays for antineutrophil cytoplasmic antibodies directed against proteinase 3 andmyeloperoxidase in Wegener's granulomatosis and microscopic polyangiitis.Rheumatology (Oxford). 2002 Nov;41(11):1313-7. PubMed PMID: 12422006.5. Braun MG, Csernok E, Gross WL, Müller-Hermelink HK. Proteinase 3, the target antigen ofanticytoplasmic antibodies circulating in Wegener's granulomatosis. Immunolocalizationin normal and pathologic tissues. Am J Pathol. 1991 Oct;139(4):831-8. PubMed PMID:1656761.6. Braun MG, Csernok E, Rögener-Schwarz W, Ludwig WD, Müller-Hermelink HK, Gross WL,et al. Monoclonal antibody WGM1 directed against proteinase 3: an immunohistochemicalmarker for naphthol ASD chloroacetate. Hematol Oncol. 1996 Jun;14(2):83-90. PubMedPMID: 8876637.7. Pederzoli M, Kantari C, Gausson V, Moriceau S, Witko-Sarsat V. Proteinase-3 inducesprocaspase-3 activation in the absence of apoptosis: potential role of thiscompartmentalized activation of membrane-associated procaspase-3 in neutrophils. JImmunol. 2005 May 15;174(10):6381-90. PubMed PMID: 15879139.
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For research and in vitro use only. Not for diagnostic or therapeutic work.Material Safety Datasheets are available at www.acris-antibodies.com or on request.
Acris Antibodies is now part of the OriGene family. Learn more at www.origene.com
MS/20180409
2 / 2