| Field | Specification |
|---|---|
| Mfr No | |
| Enzyme Type | |
| Expression System | |
| Form | Liquid |
| Formulation | |
| Product Type | |
| Purity | |
| Source | Sf9 insect cells. |
| Species | |
| Storage | |
| Target |
Product Description
Dihydrolipoamide S-Succinyltransferase Human Recombinant
Overview
DLAT gene encodes component E2 of the multi-enzyme pyruvate dehydrogenase complex (PDC). PDC is located in the inner mitochondrial membrane and catalyzes the conversion of pyruvate to acetyl coenzyme A. The protein product of this gene, dihydrolipoamide acetyltransferase, takes acetyl groups created by the oxidative decarboxylation of pyruvate and transfers them to coenzyme A. Dihydrolipoamide acetyltransferase is the antigen for antimitochondrial antibodies which are found in about 95% of patients with the autoimmune liver disease primary biliary cirrhosis (PBC). In patients who suffer from this illness, activated T lymphocytes attack and destroy epithelial cells in the bile duct where this protein is abnormally distributed and overexpressed. PBC ultimately leads to cirrhosis and liver failure. Mutations in DLAT are also a cause of pyruvate dehydrogenase E2 deficiency which causes primary lactic acidosis in infancy and early childhood.
Specifications
| Purity | DLAT purity was found to be greater than 75% as determined by SDS-PAGE. |
|---|---|
| Formulation | DLAT is supplied in 20mM HEPES buffer pH-8.0, 200mM NaCl and 20% glycerol. |
| Storage | Store DLAT at 4°C if entire vial will be used within 2-4 weeks. Store, frozen at -20°C for longer periods of time. Avoid multiple freeze-thaw cycles. |
| Expression System | Sf9 insect cells. |
