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DLAT Protein (OOPA00099)

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Gene Symbol:
Alias Symbols:
Dihydrolipoamide S-Acetyltransferase, PDC-E2, dihydrolipoyllysine-residue acetyltransferase component of pyruvate dehydrogenase complex mitochondrial, Pyruvate dehydrogenase complex component E2, 70 kDa mitochondrial autoantigen of primary biliary cir
Sf9 insect cells.
DLAT purity was found to be greater than 75% as determined by SDS-PAGE.
DLAT purity was found to be greater than 75% as determined by SDS-PAGE.
Application Info:
Western blot with anti-M2-Antigen autoantibody-positive patient sera or monoclonal anti-hexa-His-tag antibody.
DLAT (0.91mg/ml) is supplied in 20mM HEPES buffer pH-8.0, 200mM NaCl and 20% glycerol.
Product Introduction: 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.
Product Description: DLAT is a Full-length cDNA coding for the mature form of the human PDC-E2 protein having a molecular mass of 60,630 Dalton (pH 5.8). DLAT protein is fused to a hexa-histidine purification tag.
Printable datasheet for OOPA00099

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