|Product Overview:||Recombinant Human DLAT(Gln374~Trp631) fused with His tag at N-terminal was expressed in E. coli.|
|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.|
|Form:||PBS, pH7.4, containing 0.01% SKL, 1mM DTT, 5% Trehalose and Proclin300|
|Endotoxin:||<1.0EU per 1µg (determined by the LAL method)|
|Applications:||Positive Control; Immunogen; SDS-PAGE; WB.
If bio-activity of the protein is needed, please check active protein
|Stability:||The thermal stability is described by the loss rate. The loss rate was determined by accelerated thermal degradation test, that is, incubate the protein at 37 centigrade for 48h, and no obvious degradation and precipitation were observed. The loss rate is less than 5% within the expiration date under appropriate storage condition.|
|Storage:||Avoid repeated freeze/thaw cycles. Store at 2-8 centigrade for one month. Aliquot and store at -80 centigrade for 12 months.|
|Reconstitution:||Reconstitute in PBS (pH7.4) to a concentration of 0.1-1.0 mg/mL. Do not vortex|
|Gene Name:||DLAT dihydrolipoamide S-acetyltransferase [ Homo sapiens ]|
|Synonyms:||DLAT; dihydrolipoamide S-acetyltransferase; DLTA; dihydrolipoyllysine-residue acetyltransferase component of pyruvate dehydrogenase complex, mitochondrial; E2 component of pyruvate dehydrogenase complex; PDC E2; PBC; M2 antigen complex 70 kDa subunit; pyruvate dehydrogenase complex component E2; 70 kDa mitochondrial autoantigen of primary biliary cirrhosis; dihydrolipoamide acetyltransferase component of pyruvate dehydrogenase complex; dihydrolipoyllysine-residue acetyltransferase component of pyruvate dehydrogenase complex mitochondrial; PDCE2; PDC-E2;|
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