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

Polyclonal

IgG

Rabbit

The antibody is a rabbit polyclonal antibody raised against DLAT. It has been selected for its ability to recognize DLAT in immunohistochemical staining andwestern blotting.

Affinity Chromatography

Supplied as solution form in PBS, pH7.4, containing 0.02% NaN3,50% glycerol.

200ug/ml (lot specific)

Store at 4 degree C for frequent use. Stored at -20 degree C to -80 degree C in a manual defrost freezer for one year without detectable loss of activity. Avoid repeated freeze-thaw cycles.

Manufactured in an ISO 9001:2008 and ISO 13485:2003 Certified Laboratory.

Manufactured in a lab with traceable raw materials. Bulk orders can typically be prepared to the customera??s specifications, please inquire.

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.

Immunocytochemistry (ICC), Immunohistochemistry (IHC) - Formalin/Paraffin, ELISA (EIA), Western Blot (WB)

Western blotting: 1:100-400
Immunocytochemistry in formalin fixed cells: 1:100-500
Immunohistochemistry in formalin fixed frozen section: 1:100-500
Immunohistochemistry in paraffin section: 1:50-200
Enzyme-linked Immunosorbent Assay: 1:100-200

Western Blot: Sample: Recombinant protein.

68,997 Da

dihydrolipoamide S-acetyltransferase

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]

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

Chromosomal Location of Human Ortholog: 11q23.1

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

Molecular Function: dihydrolipoyllysine-residue acetyltransferase activity; protein binding

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

Disease: Pyruvate Dehydrogenase E2 Deficiency

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