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Rabbit anti-Human DLAT Monoclonal Antibody

Cat NoBMA-1514
Conjugate
Type重组兔多抗
SourceRabbit
Size50 uL
ApplicationImmunogen
FormatLiquid
ConcentrationPlease refer to the vial lable for the specific concentration.
BufferSupplied in PBS with 0.02% sodium azide,0.05% BSA,50% glycerol,pH7.3.
SpeciesHuman
StorageStore at -20℃. Avoid freeze / thaw cycles.
SynonymsDLTA;PDC-E2;PDCE2
PurificationAffinity purification
MolecularWeight70KDa
Description
BackgroundThis 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.
We can offer labeled proteins/antibodies using a broad range of intensely fluorescent dyes and labels including FITC, Biotin, Alexa Fluor, Rhodamine B, Cy, Co-Au.
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