Human Serpin A1/ alpha1-Antitrypsin Alexa Fluor™ Plus 647-conjugated Antibody
R&D Systems, part of Bio-Techne | Catalog # FAB1268AFP647
Key Product Details
Species Reactivity
Applications
Label
Antibody Source
Product Specifications
Immunogen
Specificity
Clonality
Host
Isotype
Applications
Immunocytochemistry
Immunohistochemistry
Immunoprecipitation
Western Blot
Formulation, Preparation, and Storage
Formulation
Shipping
Stability & Storage
Background: Serpin A1/alpha 1-Antitrypsin
Serpin A1 is the archetypal member of the Serpin superfamily of the serine protease inhibitors (1). As one of the most abundant proteinase inhibitors in the circulation, it is synthesized in the liver and secreted into the bloodstream with the major function to protect tissues against neutrophil elastase. A severe Serpin A1 deficiency leads to several clinical complications such as pulmonary emphysema, juvenile hepatitis, cirrhosis, and hepatocellular carcinoma (2). The deficiency is caused by point mutations in naturally occurring Serpin A1 variants (over 70 are known). For example, the Z variant (Glu342 to Lys) forms intracellular inclusion bodies, is not secreted, and leads to a severe Serpin A1 deficiency (3).
References
- Silverman, G.A. et al. (2001) J. Biol. Chem. 276:33293.
- Barbour, K.W. et al. (2002) Genomics 80:515.
- Lomas, D.A. et al. (2002) Biochem. Soc. Trans. 30:89.
Alternate Names
Entrez Gene IDs
Gene Symbol
UniProt
Additional Serpin A1/alpha 1-Antitrypsin Products
Product Specific Notices
This product is provided under an intellectual property license from Life Technologies Corporation. The transfer of this product is conditioned on the buyer using the purchased product solely in research conducted by the buyer, excluding contract research or any fee for service research, and the buyer must not (1) use this product or its components for (a) diagnostic, therapeutic or prophylactic purposes; (b) testing, analysis or screening services, or information in return for compensation on a per-test basis; or (c) manufacturing or quality assurance or quality control, and/or (2) sell or transfer this product or its components for resale, whether or not resold for use in research. For information on purchasing a license to this product for purposes other than as described above, contact Life Technologies Corporation, 5781 Van Allen Way, Carlsbad, CA 92008 USA or outlicensing@thermofisher.com.
For research use only