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Protein Catalog >> Enzymes >> DLAT Human
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Dihydrolipoamide S-Acetyltransferase Human Recombinant
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Catalogue Number ENZ-082
Synonyms 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 cirrhosis, DLAT, PBC, M2 antigen complex 70 kDa subunit, EC 2.3.1.12, EC 2.3.1.
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.
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.
Source Sf9 insect cells.
Physical Appearance Sterile Filtered colorless solution.
Formulation DLAT (0.91mg/ml) is supplied in 20mM HEPES buffer pH-8.0, 200mM NaCl and 20% glycerol.
Storage Conditions 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.
Purity DLAT purity was found to be greater than 75% as determined by SDS-PAGE.
Usage Prospec's products are furnished for LABORATORY RESEARCH USE ONLY. The product may not be used as drugs, agricultural or pesticidal products, food additives or household chemicals.
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