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

Polyclonal

Rabbit

Affinity Purified

Liquid. Purified antibody supplied in 1x PBS buffer with 0.09% (w/v) sodium azide and 2% sucrose.

For short term use, store at 2-8 degree C up to 1 week. For long term storage, store at -20 degree C in small aliquots to prevent freeze-thaw cycles.

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.

This is a rabbit polyclonal antibody against DLAT. It was validated on Western Blot using a cell lysate as a positive control.

Target Description: DLAT is dihydrolipoamide acetyltransferase, the E2 subunit of the mammalian pyruvate dehydrogenase complex of the inner mitochondrial membrane. Patients with primary biliary cirrhosis show autoantibodies to DLAT.The DLAT gene encodes dihydrolipoamide acetyltransferase (EC 2.3.1.12), the E2 subunit of the mammalian pyruvate dehydrogenase complex (PDC; EC 1.2.4.1) of the inner mitochondrial membrane. Patients with primary biliary cirrhosis (PBC; MIM 109720) show autoantibodies to DLAT.[supplied by OMIM]. Publication Note: This RefSeq record includes a subset of the publications that are available for this gene. Please see the Entrez Gene record to access additional publications. PRIMARYREFSEQ_SPAN PRIMARY_IDENTIFIER PRIMARY_SPAN COMP 1-2696 AK057299.1 1-2696 2697-3933 BC039084.1 2066-3302 3934-4322 DB551427.1 98-486

Polyclonal; Drugs and Drug Metabolism; Disease Related; Tissue Specific & Cell Marker;

Western Blot (WB)

WB Suggested Anti-DLAT Antibody Titration: 0.2-1 ug/ml
ELISA Titer: 1:62500
Positive Control: HepG2 cell lysateDLAT is strongly supported by BioGPS gene expression data to be expressed in Human HepG2 cells

69kDa

dihydrolipoamide S-acetyltransferase

dihydrolipoyllysine-residue acetyltransferase component of pyruvate dehydrogenase complex, mitochondrial

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]

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