Reactive species
Human,Mouse,Rat
Immunogen
A synthetic peptide corresponding to a sequence within amino acids 548-647 of human DLAT
(P10515).
Dilution rate
WB 1:500-2000
Purification process
Affinity purification
Stockpile
Store at -20℃. Avoid freeze / thaw cycles.
Buffer: PBS with 0.02% sodium azide,50% glycerol,pH7.3.
Other name
E2; PBC; DLTA; PDCE2; PDC-E2; DLAT
Background
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.
