DLAT Monoclonal Antibody (20 μl)

DLAT Monoclonal Antibody (20 μl)

Cat. #: 2417S
Availability: In Stock
$119.00
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Product Category:   Knockdown Validated Monoclonal Antibody
Synonyms:   Dihydrolipoamide S-Acetyltransferase; Dihydrolipoyllysine-Residue Acetyltransferase Component Of Pyruvate Dehydrogenase Complex, Mitochondrial; Dihydrolipoamide Acetyltransferase Component Of Pyruvate Dehydrogenase Complex; 70 KDa Mitochondrial Autoantigen Of Primary Biliary Cirrhosis; E2 Component Of Pyruvate Dehydrogenase Complex; Pyruvate Dehydrogenase Complex Component E2; M2 Antigen Complex 70 KDa Subunit; EC 2.3.1.12; PDC-E2; PDCE2; DLTA; PBC; Dihydrolipoyllysine-Residue Acetyltransferase; EC 2.3.1; E2
UniProt Entry:    P10515
NCBI Gene Entry:   1737
Molecular Weight:   69 kDa
Clonality:   Mouse monoclonal antibody
Species Reactivity:   Human
Applications:   WB
Recommended Dilution:   WB 1:1000
Immunogen:   Recombinant fragment of human DLAT
Isotype:   Mouse IgG 
Storage Buffer:   Supplied in PBS (pH 7.5) containing 50 µg/ml BSA, 50% glycerol, and 0.01% sodium azide  
Storage:   At –20°C
Background:   DLAT 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.
Documents: Please contact us to request the manual

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