PR3 (PRTN3) Mouse Monoclonal Antibody [Clone ID: PR3-G2]

Specifications

Product Data
Clone Name	PR3-G2
Applications	ELISA, IHC, WB
Recommend Dilution	Immunohistochemistry on frozen section: The typical starting working dilution is 1:50. Flow cytometry: The typical starting working dilution is 1:50. Immunoassay. Western blot: The typical starting working dilution is 1:50.
Reactivity	Human
Host	Mouse
Clonality	Monoclonal
Immunogen	A crude granule extract
Specificity	Monoclonal antibody PR3G-2 reacts with human proteinase 3 (PR3), a 30 kDa protein.
Formulation	PBS Label: FITC State: Liquid 0.2 µm filtered Ig fraction Stabilizer: 1% bovine serum albumin Preservative: 0.02% sodium azide
Concentration	lot specific
Purification	Protein G
Conjugation	FITC
Storage Condition	Store at 2 - 8 °C.
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 (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.
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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