DLAT Rabbit monoclonal antibody
Product Sizes
5 ul
£ POA
A8814-5UL
20 ul
£163.00
A8814-20UL
100 ul
£342.00
A8814-100UL
500 ul
£1532.00
A8814-500UL
1000 ul
£2704.00
A8814-1000UL
About this Product
- SKU:
- A8814
- Additional Names:
- DLAT|DLTA|E2|PBC|PDC-E2|PDCE2
- Application:
- ELISA, Western Blot
- Clonality:
- Monoclonal
- Extra Details:
- 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.
- Formulation:
- Unmodified
- Host:
- Rabbit
- Immunogen:
- Synthetic peptide. This information is considered to be commercially sensitive.
- Isotype:
- IgG
- Molecular Weight:
- 69 kDa
- Purification:
- Affinity Purified
- Reactivities:
- Human, Mouse, Rat
- Sequence:
- AREGKLQPHEFQGGTFTISNLGMFGIKNFSAIINPPQACILAIGASEDKLVPADNEKGFDVASMMSVTLSCDHRVVDGAVGAQWLAEFRKYLEKPITMLL
- Shipping Conditions:
- Blue Ice
- Storage Conditions:
- -20[o]C Avoid freeze/thaw cycles.
- Supplier:
- ABclonal Technology
- Type:
- Antibody: Monoclonal Antibody
- Manufacturer's Data Sheet:A8814
