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Merck

DLAT

dihydrolipoamide S-acetyltransferase

Synonyms:
DLTA, PDC-E2, PDCE2
Species:
Human DLAT (1737), Mouse Dlat (235339), Rat Dlat (81654), chicken DLAT (419796), Zebrafish dlat (324201), domestic guinea pig Dlat (100735563), dog DLAT (489406), sheep DLAT (101110965), cow DLAT (512723), Horse DLAT (100062027) ...More
UniProtKB ID:
P10515, Q16783, Q53EP3
Gene ID:
  • Human(1737) Summary: 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.[provided by RefSeq, Oct 2009]
  • Mouse(235339) dihydrolipoamide S-acetyltransferase (E2 component of pyruvate dehydrogenase complex)
  • Rat(81654) dihydrolipoamide S-acetyltransferase
  • chicken(419796) dihydrolipoamide S-acetyltransferase
  • Zebrafish(324201) dihydrolipoamide S-acetyltransferase (E2 component of pyruvate dehydrogenase complex)
  • domestic guinea pig(100735563) dihydrolipoamide S-acetyltransferase
  • dog(489406) dihydrolipoamide S-acetyltransferase
  • sheep(101110965) dihydrolipoamide S-acetyltransferase
  • cow(512723) dihydrolipoamide S-acetyltransferase
  • Horse(100062027) dihydrolipoamide S-acetyltransferase
  • domestic cat(101094126) dihydrolipoamide S-acetyltransferase
  • Domestic Rabbit(100349212) dihydrolipoamide S-acetyltransferase
  • naked mole-rat(101698667) dihydrolipoamide S-acetyltransferase

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Antibodies

Product Number
Description
Species Reactivity
Application
AV41994
Anti-DLAT antibody produced in rabbit, IgG fraction of antiserum,
Species Reactivity
bovine, dog, guinea pig, horse, human, mouse, rabbit, rat
Application
immunohistochemistry
western blot