Alpha-1-antitrypsin, Human Antibody from HYCULT BIOTECH

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Antigenic SpecificityAlpha-1-antitrypsin, Human
Clonepolyclonal
Host SpeciesGoat
Reactive Specieshuman
Isotypen/a
FormatProtein G purified
Size20 µg
Concentrationn/a
ApplicationsImmuno assays, Western blot. RUO
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DescriptionThe goat polyclonal antibody recognizes human alpha-1-antitrypsin. This protein 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.
ImmunogenMonomeric Z AAT purified from donor plasma
Other NamesAlpha-1 protease inhibitor, Alpha-1-antiproteinase, Serpin A1 Gene name: SERPINA1, AAT, PI
Gene, Accession #n/a
Catalog #HP9063-20UG
Price$157
Order / More InfoAlpha-1-antitrypsin, Human Antibody from HYCULT BIOTECH
Product Specific Referencesn/a
HYCULT BIOTECH
HYCULT BIOTECH
HYCULT BIOTECH
Frontstraat 2a
5405 PB Uden THE NETHERLANDS
P: +31 (0)413 251335
F: +31 (0)413 248353

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