dihydrolipoamide S-acetyltransferase; DLAT; DLTA; PDC-E2; PDCE2

For Research Use Only. Not for use in diagnostic procedures.

Polyclonal

IgG

Rabbit

Antigen Affinity Purified

Manufactured in an ISO 13485:2003 and EN ISO 13485:2012 Certified Laboratory.

Small volumes of anti-DLAT antibody vial(s) may occasionally become entrapped in the seal of the product vial during shipment and storage. If necessary, briefly centrifuge the vial on a tabletop centrifuge to dislodge any liquid in the container`s cap. Certain products may require to ship with dry ice and additional dry ice fee may apply.

The relevance of mitochondrial dysfunction in human disease is an increasingly important topic, as judged by the number of recent Pubmed articles. Human dihydrolipoamide acetyltransferase is a critical component of the pyruvate dehydrogenase complex - TCA Cycle - in the outer membrane and mutations in the DLAT gene can result in metabolism disorders, while the acetyltransferase component is highly antigenic and may lead to autoantigen primary biliary cirrhosis (PBC). Thus, research into both mechanism and drug discovery may provide clues to correct function of this E2 component pyruvate dehydrogenase activity. A rabbit polyclonal DLAT antibody has been validated for use in ELISA and western blot cell applications, has broad reactivity, excellent protein specificity and can detection low expression. Compared to mouse products, this polyclonal antibody recognizes a 70 kDa product and results are included with specific loading control on datasheet for antibodies. Suggested uses include metabolic process experimentation.

ELISA (EIA), Western Blot (WB)

68,997 Da[Similar Products]

dihydrolipoamide S-acetyltransferase

dihydrolipoyllysine-residue acetyltransferase component of pyruvate dehydrogenase complex, mitochondrial; PBC; M2 antigen complex 70 kDa subunit; pyruvate dehydrogenase complex component E2; E2 component of pyruvate dehydrogenase complex; 70 kDa mitochondrial autoantigen of primary biliary cirrhosis; dihydrolipoamide acetyltransferase component of pyruvate dehydrogenase complex; dihydrolipoyllysine-residue acetyltransferase component of pyruvate dehydrogenase complex mitochondrial

Dihydrolipoyllysine-residue acetyltransferase component of pyruvate dehydrogenase complex, mitochondrial

DLTA; PBC; PDC-E2; PDCE2??[Similar Products]

ODP2_HUMAN

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.[provided by RefSeq, Oct 2009]

DLAT: the E2 component of the pyruvate dehydrogenase complex (PDHC) that catalyzes the overall conversion of pyruvate to acetyl-CoA and CO2. Contains two lipoyl-binding domains. The PDHC plays a major role in controlling the balance between lipid and glucose oxidation depending on substrate availability. The activity of the PDHC is tightly regulated by phosphorylation of the E1 components by the PDHKs. The pyruvate dehydrogenase (PDH) holoenzyme is a multi-enzyme complex (PDHC) that contains 20-30 copies of pyruvate decarboxylase tetramers (2 alpha:2 beta)(E1), 60 copies of dihydrolipoamide acetyltransferase (E2), six homo-dimers of dihydrolipoamide dehydrogenase (E3), plus E3 binding proteins. PDHKs are recruited to the PDHC by binding to a lipoyl group covalently attached to K174 of the inner lipoyl domain of the E2 component.

Protein type: Mitochondrial; Carbohydrate Metabolism - pyruvate; EC 2.3.1.12; Carbohydrate Metabolism - glycolysis and gluconeogenesis; Carbohydrate Metabolism - citrate (TCA) cycle; Transferase

Chromosomal Location of Human Ortholog: 11q23.1

Cellular Component: mitochondrion; mitochondrial matrix; mitochondrial pyruvate dehydrogenase complex

Molecular Function: dihydrolipoyllysine-residue acetyltransferase activity; protein binding

Biological Process: cellular metabolic process; tricarboxylic acid cycle; glucose metabolic process; regulation of acetyl-CoA biosynthetic process from pyruvate; pyruvate metabolic process

Disease: Pyruvate Dehydrogenase E2 Deficiency

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