PR3 (PRTN3) Mouse Monoclonal Antibody [Clone ID: WGM2]

Specifications

Product Data
Clone Name	WGM2
Applications	ELISA, FN, IHC, WB
Recommend Dilution	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 human PR3-ANCA to PR3. Immunohistochemistry on Frozen and Paraffin Sections.
Reactivity	Human
Host	Mouse
Clonality	Monoclonal
Specificity	This Monoclonal antibody WGM2 reacts with Human Proteinase 3 (PR3), a 30 kDa protein.
Formulation	PBS State: Purified State: Liquid 0.2 µm filtered Ig fraction Stabilizer: 0.1% BSA Preservative: None
Concentration	lot specific
Purification	Protein G Chromatography
Conjugation	Unconjugated
Storage Condition	Store undiluted at 2-8°C. DO NOT FREEZE!
Gene Name	proteinase 3
Database Link	Entrez Gene 5657 Human UniProt ID P24158
Background	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.
Synonyms	Myeloblastin, MBN, Leukocyte proteinase 3, Proteinase 3, PRTN3, PR-3, Neutrophil proteinase 4, NP-4, P29, Wegener autoantigen, AGP7
Reference Data

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