83-5980-11　Alpha-1 Antitrypsin 100ug　051952

特徴

• 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 polymericalpha-1 antitrypsin (see Ref.1).
• Western Blot: 1:50, under reducing and non-reducing conditionsOptimal dilutions to be determined by the researcher.
• Storage and Stability:May be stored at 4℃ for short-term only.
• Aliquot to avoid repeated freezing and thawing.
• Store at -20℃.
• 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.