Pyruvate Dehydrogenase E2 (DLAT) Rabbit Polyclonal Antibody
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Other products for "DLAT"
Specifications
Product Data | |
Applications | IHC, WB |
Recommended Dilution | WB: 200-1000 WB positive control: Lncap, hepg2 and A431 cell IHC: 25-100 Positive control: Human liver cancer Predicted cell location: Cytoplasm |
Reactivities | Human, Mouse, Rat |
Host | Rabbit |
Isotype | IgG |
Clonality | Polyclonal |
Immunogen | Fusion protein of human DLAT |
Formulation | pH7.4 PBS, 0.05% NaN3, 40% Glyceroln |
Concentration | lot specific |
Purification | Antigen affinity purification |
Conjugation | Unconjugated |
Storage | Store at -20°C as received. |
Stability | Stable for 12 months from date of receipt. |
Predicted Protein Size | 69 kDa |
Gene Name | dihydrolipoamide S-acetyltransferase |
Database Link | |
Background | This gene encodes component E2 of the multi-enzyme pyruvate dehydrogenase complex (PDC). PDC resides in the inner mitochondrial membrane and catalyzes the conversion of pyruvate to acetyl coenzyme A. The protein product of this gene, dihydrolipoamide acetyltransferase, accepts acetyl groups formed by the oxidative decarboxylation of pyruvate and transfers them to coenzyme A. Dihydrolipoamide acetyltransferase is the antigen for antimitochondrial antibodies. These autoantibodies are present in nearly 95% of patients with the autoimmune liver disease primary biliary cirrhosis (PBC). In PBC, activated T lymphocytes attack and destroy epithelial cells in the bile duct where this protein is abnormally distributed and overexpressed. PBC enventually leads to cirrhosis and liver failure. Mutations in this gene are also a cause of pyruvate dehydrogenase E2 deficiency which causes primary lactic acidosis in infancy and early childhood. |
Synonyms | DLTA; PDC-E2; PDCE2 |
Reference Data | |
Protein Families | Druggable Genome |
Protein Pathways | Citrate cycle (TCA cycle), Glycolysis / Gluconeogenesis, Metabolic pathways, Pyruvate metabolism |
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