Molecular weight (DA)
69kD
Immunogen
Purified recombinant human Pyruvate Dehydrogenase E2 protein fragments expressed in E.coli.
Specificity
This antibody detects endogenous levels of Pyruvate Dehydrogenase E2 and does not cross-react with related proteins.
Formulation
Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide.
Dilution rate
wb 1:1000 icc 1:300
Purification process (Immunogen)
The antibody was affinity-purified from mouse ascites by affinity-chromatography using epitope-specific immunogen.
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 lact
Function
catalytic activity:Acetyl-CoA + enzyme N(6)-(dihydrolipoyl)lysine = CoA + enzyme N(6)-(S-acetyldihydrolipoyl)lysine.,cofactor:Binds 2 lipoyl cofactors covalently.,disease:Defects in DLAT are the cause of pyruvate dehydrogenase E2 deficiency [MIM:245348]; also known as lactic acidemia due to defect of E2 lipoyl transacetylase of the pyruvate dehydrogenase complex. Pyruvate dehydrogenase (PDH) deficiency is a major cause of primary lactic acidosis and neurological dysfunction in infancy and early childhood. In this form of PDH deficiency episodic dystonia is the major neurological manifestation, with other more common features of pyruvate dehydrogenase deficiency, such as hypotonia and ataxia, being less prominent.,disease:Primary biliary cirrhosis is a chronic, progressive cholestatic liver disease characterized by the presence of antimitochondrial autoantibodies in patients' serum. It ma
Abbreviation
Pyruvate Dehydrogenase E2
Other name
70 kDa mitochondrial autoantigen of primary biliary cirrhosis;anti DLAT; Dihydrolipoamide acetyltransferase component of pyruvate dehydrogenase complex; Dihydrolipoamide;Dihydrolipoamide S Acetyltransferase;Dihydrolipoamide S-acetyltransferase (E2 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;DLAT;DLTA;E2;E2 component of pyruvate dehydrogenase complex;EC 2.3.1.12;M2 antigen complex 70 kDa subunit;M2 Antigen Complex 70kD Subunit;mitochondrial;ODP2_HUMAN;PBC;PDC E2;PDC-E2;PDCE2;Pyruvate dehydrogenase complex component E2;Pyruvate dehydrogenase complex E2 subunit;S acetyltransferase component of pyruvate dehydrogenase complex.
Fields
>>Glycolysis / Gluconeogenesis;>>Citrate cycle (TCA cycle);>>Pyruvate metabolism;>>Metabolic pathways;>>Carbon metabolism
Human protein sequence Database
P10515
Mouse protein sequence database
Q8BMF4
Rat protein sequence database
Cellular localization
Mitochondrion matrix.
Tissue expression
Heart,Keratinocyte carcinoma,Kidney,Liver,Placenta,Testis,
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