| Product Name | Anti-Pyruvate Dehydrogenase E2 antibody-RBITC |
|---|---|
| Antibody Type | Primary Antibodies |
| Immunogen | Polypeptide |
| Conjugation | RBITC |
| Clonality | Polyclonal |
|---|---|
| Isotype | IgG |
| Host Species | Rabbit |
| Species Reactivity | HumanMouseRat |
| Concentration | 1mg/ml |
| Purification | Protein A |
| Gene Symbol | DLAT |
|---|---|
| Gene Synonyms | 6332404G05Rik DLTA E2 PBC PDC-E2 PDCE2 |
| Gene Full Name | dihydrolipoamide S-acetyltransferase |
| Gene Summary | 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.[provided by RefSeq, Oct 2009] |
| Alternative Names | 70 kDa mitochondrial autoantigen of primary biliary cirrhosis anti DLAT Dihydrolipoamide dihydrolipoamide acetyltransferase component of pyruvate dehydrogenase complex dihydrolipoamide acetyltransferase PDC-E2 dihydrolipoamide S-acetyltransferase (E2 component of pyruvate dehydrogenase complex) E2 E2 component of pyruvate dehydrogenase complex EC 2.3.1.12 M2 antigen complex 70 kDa subunit ODP2 PBC PDC PDC-E2 PDC-E2 PDC-E2 component of the pyruvate dehydrogenase complex PDCE2 primary biliary cirrhosis autoantigen pyruvate dehydrogenase complex component E2 |
| Molecular Weight(MW) | 69kDa |
| Source | Rabbit |
| Cellular Localization | Mitochondrion matrix |
| SwissProt ID | P10515 |
|---|
| Form | Liquid |
|---|---|
| Storage Instructions | Shipped at 4°C. Store at +4°C short term (1-2 weeks). Store at -20°C long term. Avoid freeze / thaw cycle. |
| Storage Buffer | Purified antibody in PBS with 0.05% PC300,40% Glycerol. |
