Your shopping cart is empty!
Anti-Pyruvate Dehydrogenase E2 Rabbit pAb (100 μl)
| Reactivity: | H,R |
| Applications: | IHC/IF |
| Host Species: | Rabbit |
| Clonality: | Polyclonal |
| Gene Name: | Dihydrolipoyllysine-residue acetyltransferase component of pyruvate dehydrogenase complex, mitochondrial |
Synonyms: | DLAT, DLTA, PBC, PDC-E2, PDCE2, Pyruvate dehydrogenase complex component E2 |
Immunogen: | Recombinant protein corresponding to Mouse Pyruvate Dehydrogenase E2 |
Uniprot ID: | Q8BMF4 |
Isotype: | IgG |
Purity: | Affinity purification |
Subcellular location: | Mitochondrion |
Product Usage Information
IHC/IF | Human, Rat | 1: 500-1: 1000 | heart, kidney |
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.
Images
| Immunohistochemistry analysis of paraffin-embedded human kidney using DLAT (GB113649) at dilution of 1: 1000 |
| Immunohistochemistry analysis of paraffin-embedded rat kidney using DLAT (GB113649) at dilution of 1: 1000 |
| Immunohistochemistry analysis of paraffin-embedded rat heart using DLAT (GB113649) at dilution of 1: 1000 |
Storage
| Storage | Store at -20°C for one year. Avoid repeated freeze/thaw cycles. |
| Storage Buffer | PBS with 0.02%sodium azide,100 μg/ml BSA and 50% glycerol. |
