MB0031

antibody from Bioworld Technology, Inc
Targeting: DLAT DLTA, E2, PDC-E2
Provider product page for MB0031
Western blot
Immunoprecipitation

Antibody data

Product number
MB0031 - Provider product page
Provider
Bioworld Technology, Inc
Product name
DLAT monoclonal antibody
Antibody type
Monoclonal
Antigen
Recombinant full length Human DLAT.
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.
Reactivity
Human, Mouse
Host
Mouse
Isotype
IgG
Vial size
100ul
Concentration
1 mg/ml
Storage
Store at 4°C short term. Aliquot and store at -20°C long term. Avoid freeze-thaw cycles.
DLAT protein structure - MB0031 shown in red.