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GeneBio Systems

DLAT Polyclonal Antibody

DLAT Polyclonal Antibody

SKU:E-AB-17216

Regular price €493,95 EUR
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Size: 200μL

Storage: Store at -20℃ Valid for 12 months. Avoid freeze / thaw cycles.

Shipping: The product is shipped with ice pack,upon receipt,store it immediately at the temperature recommended.

Exp date: 12 months

Category ID_II: Primary Antibodies

Category ID_III: Polyclonal Antibodies

Abbreviation: DLAT

Target Synonym: 70 kDa mitochondrial autoantigen of primary biliary cirrhosis;Dihydrolipoamide acetyltransferase component of pyruvate dehydrogenase complex;Dihydrolipoamide S Acetyltransferase;Dihydrolipoyllysine-residue acetyltransferase component of pyruvate dehydrogenase complex;dihydrolipoyllysine-residue acetyltransferase component of pyruvate dehydrogenase complex mitochondrial;DLAT;DLTA;E2;E2 component of pyruvate dehydrogenase complex;M2 antigen complex 70 kDa subunit;mitochondrial;ODP2;PBC;PDC E2;PDC-E2;PDCE2;Pyruvate dehydrogenase complex component E2;Pyruvate dehydrogenase complex E2 subunit

Research Areas: Cancer;Metabolism;Signal transduction

Conjugation: Unconjugated

Host: Rabbit

Species reactivity: Human;Mouse;Rat

Application: IHC

Isotype: IgG

Clonality: Polyclonal

Clone NO.:

UNIProt ID: P10515

Accession:

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.

Concentration: 0.6 mg/mL

Immunogen: Fusion protein of human DLAT

Buffer: PBS with 0.05% sodium azide and 50% glycerol, PH7.4

Purification method: Affinity purification

Dilution: IHC 1:30-150

Calculated MW: 69 kDa

ObservedMW:

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