P9099-01B
Proteinase 3 (PR3) (FITC)
Size
£1,266.00
- SKU:
- P9099-01B
- Purity:
- Purified by Protein G affinity chromatography.
- Storage Conditions:
- -20°C
- Supplier:
- United States Biological
- Host:
- Mouse
- Immunogen:
- Neutrophils (degranulated supernatant)
- Clone:
- WGM2
- Format:
- Supplied as a liquid in PBS, 1% BSA, 0.02% sodium azide. Labeled with Fluorescein isothiocyanate (FITC).
- Extra Details:
- 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. WGM2 blocks the PR3 activity and partially inhibits the binding of human PR3-ANCA to PR3. Applications: Suitable for use in Functional Studies, Flow Cytometry, Western Blot, Immunoassays and Immunohistochemistry. Other applcations not tested. Recommended Dilutions: Immunohistochemistry (frozen and paraffin): 1:50 Flow Cytometry: 1:50 Western Blot: 1:50 Functional Studies: inhibition of biological PR3 activity and induction of monocytes and neutrophils activation. Optimal dilutions to be determined by the researcher. Storage and Stability: Store product at 4°C if to be used immediately within two weeks. For long-term storage, aliquot to avoid repeated freezing and thawing and store at -20°C. Aliquots are stable at -20°C for 12 months after receipt. Dilute required amount only prior to immediate use. Further dilutions can be made in assay buffer. Caution: FITC conjugates are sensitive to light. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap.
- Shipping Conditions:
- Blue Ice