DLAT Antilichaam

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.

1737

P10515

Human, Mouse, Rat

Recombinant fusion protein containing a sequence corresponding to amino acids 87-270 of human DLAT (NP_001922.2) .

Unconjugated

Primary Antibodies

Cancer, Signal Transduction

Affinity purification

HeLa

Batch dependent

PBS with 0.02% sodium azide, 50% glycerol, pH7.3.

None

Blue Ice

Store at -20˚ C. Avoid freeze / thaw cycles.

Observed: 69kDa

IgG

DLAT

MCF7

HepG2

K-562

Mouse brain

Rat brain

Dihydrolipoamide S-acetyltransferase

Homo sapiens

DLAT, DLTA, PDC-E2, PDCE2

WB, IHC

Liquid