PR3 / C-ANCA antibody

Principal name

PR3 / C-ANCA antibody

Alternative names for PR3 / C-ANCA antibody

Myeloblastin, MBN, Leukocyte proteinase 3, Proteinase 3, PRTN3, PR-3, Neutrophil proteinase 4, NP-4, P29, Wegener autoantigen, AGP7

SwissProt ID

P24158 (Human), Q61096 (Mouse), Q9UQD8 (Human)

Gene ID

5657 (PRTN3), 19152 (Prtn3)

Available reactivities

Hu (Human), Ms (Mouse)

Available hosts

Mouse, Rabbit, Goat

Available applications

Enzyme Immunoassay (E), Western blot / Immunoblot (WB), ELISA (detection) (E(detection)), Frozen Sections (C), Flow Cytometry (F), Functional assay (FN), Paraffin Sections (P)

Background of PR3 / C-ANCA antibody

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

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Primary Antibodies

Catalog No. Host Iso. Clone Pres. React. Applications  

PR3 / C-ANCA antibody

PR3 / C-ANCA Mouse IgG1 WGM2 FITC Hu C, E, FN, P, WB
0.1 mg / €560.00
  Acris Antibodies GmbH

PR3 / C-ANCA antibody

PR3 / C-ANCA Mouse IgG1 WGM2 Purified Hu C, E, FN, P, WB
0.1 mg / €440.00
  Acris Antibodies GmbH

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