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Gene Bio Systems

DLAT Antibody - Cat. #: CSB-PA084734

DLAT Antibody - Cat. #: CSB-PA084734

SKU:CSB-PA084734

Regular price $416.00 USD
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Size :50ul

Clone Number:

Aliases:70 kDa mitochondrial autoantigen of primary biliary cirrhosis antibody; Dihydrolipoamide acetyltransferase component of pyruvate dehydrogenase complex antibody; Dihydrolipoamide S Acetyltransferase antibody; Dihydrolipoyllysine-residue acetyltransferase component of pyruvate dehydrogenase complex antibody; dihydrolipoyllysine-residue acetyltransferase component of pyruvate dehydrogenase complex mitochondrial antibody; DLAT antibody; DLTA antibody; E2 antibody; E2 component of pyruvate dehydrogenase complex antibody; M2 antigen complex 70 kDa subunit antibody; mitochondrial antibody; ODP2_HUMAN antibody; PBC antibody; PDC E2 antibody; PDC-E2 antibody; PDCE2 antibody; Pyruvate dehydrogenase complex component E2 antibody; Pyruvate dehydrogenase complex E2 subunit antibody

Product Type:Polyclonal Antibody

Immunogen Species:Homo sapiens (Human)

UniProt ID:P10515

Immunogen:Fusion protein of Human DLAT

Raised in:Rabbit

Species Reactivity:Human, Mouse, Rat

Tested Applications:ELISA, WB, IHC; ELISA:1:2000-1:5000, WB:1:500-1:2000, IHC:1:30-1:150

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.

Clonality:Polyclonal

Isotype:IgG

Purification Method:Antigen affinity purification

Conjugate:Non-conjugated

Buffer:-20°C, pH7.4 PBS, 0.05% NaN3, 40% Glycerol

Form:Liquid

Stroage:Upon receipt, store at -20°C or -80°C. Avoid repeated freeze.

Target Names:DLAT

Research Areas:Cancer;Metabolism;Signal transduction

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