DLAT polyclonal antibody
Product Specifications
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.
CAS Number
9007-83-4
Structure Composition
1mg/ml in PBS with 0.02% sodium azide, 50% glycerol, pH7.2
Product Name Alternative
DLAT; DLTA; PDC-E2; PDCE2
Swiss Prot
P10515
Reactivity
Human, Mouse, Rat
Immunogen
Recombinant fusion protein of human DLAT (NP_001922.2) .
Conjugation
Unconjugated
Applications
WB, IHC, IF/ICC
Dilution
WB,1:500 - 1:2000|IHC,1:50 - 1:100|IF/ICC,1:50 - 1:200
Purity
The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen and the purity is > 95% (by SDS-PAGE) .
Modification
Unmodification
Molecular Weight
69kDa
Storage Conditions
Store at 4°C short term. Aliquot and store at -20°C long term. Avoid freeze-thaw cycles.
Notes
For research use only, not for use in diagnostic procedure.
Specificity
Unmodification
Host or Source
Rabbit
Available Sizes
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