Alpha-1-antitrypsin, Human Antibody from HYCULT BIOTECH

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Antigenic SpecificityAlpha-1-antitrypsin, Human
Clone2C1
Host SpeciesMouse
Reactive Specieshuman
IsotypeIgG1
Formatunconjugated
Size500 µg
Concentrationn/a
ApplicationsImmuno assays, Immuno fluorescence, Immuno precipitation, Paraffin sections, Western blot. RUO
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DescriptionThe mouse monoclonal antibody clone 2C1 recognizes polymeric forms of human alpha-1-antitrypsin. Alpha-1-antitrypsin is the most abundant circulating protease inhibitor. Serpinopathies are conformational diseases characterized by the polymerization and intracellular retention of members of the serine protease inhibitor or serpin superfamily of proteins.1 The best known is a1-antitrypsin deficiency, with the most common severe deficiency allele being the Z mutation (Glu342Lys). The severe Z deficiency allele (Glu342Lys) causes the protein to undergo a conformational transition and form ordered polymers that are retained within hepatocytes. This causes neonatal hepatitis, cirrhosis, and hepatocellular carcinoma. Clone 2C1 recognizes polymers formed by Z α1-antitrypsin in vivo. It also recognizes polymers formed by the Siiyama (Ser53Phe) and Brescia (Gly225Arg) mutants, and the novel His334Asp shutter domain mutant of a1-antitrypsin that is associated with prolonged neonatal jaundice in a 6-week-old boy. These data show that Z and shutter domain mutants form polymers with a shared epitope.
ImmunogenZ a1-antitrypsin polymers prepared from a1-antitrypsin purified from the plasma of PI*Z homozygotes
Other NamesAlpha-1 protease inhibitor, Alpha-1-antiproteinase, Serpin A1, A1AT Gene name: SERPINA1
Gene, Accession #n/a
Catalog #HM2289-500UG
Price$1468
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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