DLAT Rabbit mAb
$148.00 – $548.00
E2; PBC; DLTA; PDCE2; PDC-E2
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| Catalog No. | A8814 |
|---|---|
| Product Name | DLAT Rabbit mAb |
| Supplier Name | ABclonal, Inc. |
| Brand Name | Abclonal |
| Synonyms | E2; PBC; DLTA; PDCE2; PDC-E2 |
| Gene Name | DLAT |
| Protein Name | DLAT |
| Uniprot/Swissprot ID | P10515 |
| Gene ID | 1737 |
| Clone | ARC1314 |
| Clonality | Monoclonal |
| Source/Host | Rabbit |
| Reactivity | Human, Mouse, Rat |
| Conjugate | Unconjugated |
| Note | Products will be shipped from the warehouse in Massachusetts. Promotion is running from time to time. Welcome to send a request for quote to message@sydlabs.com. |
| Order Offline | Syd Labs, Inc. 4 Avenue E, Hopkinton, MA 01748 USA. Phone: 1-617-401-8149 Fax: 1-617-606-5019 Email: message@sydlabs.com |
Description
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.
