051952
Alpha-1 Antitrypsin
Size
£1,068.00
- SKU:
- 051952
- Purity:
- Purified by Protein G affinity chromatography.
- Storage Conditions:
- -20°C
- Supplier:
- United States Biological
- Host:
- Mouse
- Immunogen:
- Monomeric Z a1-antitrypsin purified from the plasma of a PI*ZZ homozygote
- Clone:
- 3C11
- Format:
- Supplied as a liquid in PBS, 0.1% BSA, 0.02% sodium azide.
- Extra Details:
- Alpha-1-antitrypsin is a member of the serine protease inhibitor (serpin) superfamily which are proteins known for their ability to inhibit proteases. It is the most abundant circulating protease inhibitor known. It mainly targets enzymes released by neutrophils, especially neutrophil elastase (NE) but also proteinase 3 (PR3) and Cathepsin G (CG). Serpinopathies are conformational diseases characterized by the polymerization and intracellular retention of members of the serpin superfamily. The best known is alpha-1 antitrypsin deficiency, with the most common severe deficiency allele being the Z mutation (Glu342Lys). This severe autosomal dominant disorder causes the protein to undergo a conformational transition and form ordered polymers that are retained within hepatocytes. Due to this accumulation of polymers in hepatocytes, blood alpha-1 trypsin levels will decrease leading to chronic uninhibited tissue breakdown. This causes the degradation especially of lung tissue which will eventually lead to pulmonary emphysema. In addition, accumulation of polymers in hepatocytes causes liver diseases such as neonatal hepatitis, cirrhosis, and hepatocellular carcinoma. Applications: Suitable for use in ELISA and Western Blot. Other applications not tested. Recommended Dilutions: ELISA: 1:50. When used as a detection antibody in sandwich ELISA, 051952 reacts with similar affinity with monomeric and polymeric alpha-1 antitrypsin (see Ref.1). Western Blot: 1:50, under reducing and non-reducing conditions Optimal dilutions to be determined by the researcher. Storage and Stability: May be stored at 4°C for short-term only. Aliquot to avoid repeated freezing and thawing. Store at -20°C. Aliquots are stable for 12 months after receipt. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap.
- Shipping Conditions:
- Blue Ice