Article No
182-HM2289-100UG
Alpha-1-antitrypsin, Human, mAb 2C1 – 100 µg
Alpha-1-antitrypsin, Human, mAb 2C1 – 100 µg
Antibodies Primary
Specifications
| Application | IA, IF, IP, WB |
| Article No | 182-HM2289-100UG |
| Country Availability | SE, FI, DK, NO, EE, LV, LT |
| Clone | 2C1 |
| Clone Type | monoclonal |
| Conjugation | Unconjugated |
| Description | Alpha-1-antitrypsin, Human, mAb 2C1 – 100 µg |
| Supplier | Hycult biotechnology |
| Format | 1 ml (100 μg/ml) 0.2 μm filtered protein G purified antibody solution in PBS, containing 0.1% bovine serum albumin and 0.02% sodium azide. |
| Immunogen | Z a1-antitrypsin polymers prepared from a1-antitrypsin purified from the plasma of PI*Z homozygotes |
| Isotype | IgG1 |
| Notes | The 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. |
| Alias Names | Alpha-1 protease inhibitor, Alpha-1-antiproteinase, Serpin A1, A1AT Gene name: SERPINA1 |
| Previous Article No | 182-HM2289, 182-HM2289-100UG, HM2289-100UG |
| Product Type | Antibodies Primary |
| Research area | Immunology |
| Shipping Information | Room Temperature |
| Size | 100 µg |
| Source / Host | mouse |
| Species Reactivity | human |
| Storage | Product should be stored at 4°C. Under recommended storage conditions, product is stable for at least one year. |
| Substrate / Buffer | Quantities >500 µg will be send without preservative and carrier free. Quantities |
| Product Page Updated | 2024-04-05T09:42:44.568Z |
