Anti-Pyruvate Dehydrogenase E2 Mouse mAb

Antibody type:Primary antibody

Label:Unconjugated

Modification:Unmodification

Isotype:IgG1

Host:Mouse

Application:WB,ICC,IP

Purify method:Affinity purified

Species:Human,Mouse

Gene Name:dlat

Synonyms:DLTA; PDCE2; PDC-E2

Gene Synonyms: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 compone

Gene Full Name:

Gene Infomation:

Antigen:Purified recombinant human Pyruvate Dehydrogenase E2 protein fragments expressed in E.coli.

Antigen Synonyms:

Clonality:Monclonal antibody

Source:Human

Reaction:This antibody detects endogenous levels of Pyruvate Dehydrogenase E2 and does not cross-react with related proteins.

Form:Liquid

Tested Applications:

• Western blot (1:100 to 1:500)
• Immunofluorescence (1:50 to 1:400)
• Immunohistochemistry (1:200 to 1:500)
• Flow cytometry analysis (1:200 to 1:500)
• Enzyme-linked Immunosorbent Assay (1:100-1:5000)

Note: Users are strongly advised to determine the optimal dilution of antibody to use for their specific applications.

Clone:4A4-B6-C10

Dilution:WB 1:1000,ICC 1:300

Mole Mass:69kDa

Location:Mitochondrion matrix.

Concentration:

Sequence Similarity:Belongs to the 2-oxoacid dehydrogenase family._x000D__x000D_Contains 2 lipoyl-binding domains.

Gene Id:1737

SwissProt ID:P10515

Unigene:

Nucleotide Accession:

Tissue specificity:

Storage:Store at +4°C short term. Store at -20°C long term. Avoid freeze / thaw cycle.

Buffer condition:Purified mouse monoclonal in buffer containing 0.1M Tris-Glycine (pH 7.4 150 mM NaCl) with 0.02% sodium azide 50% glycerol

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.

Molar Function:

Western Blot: 

Stability: The thermal stability is described by the loss rate. The loss rate was determined by accelerated thermal degradation test, that is, incubate the protein at 37°C for 48h, and no obvious degradation and precipitation were observed. The loss rate is less than 5% within the expiration date under appropriate storage condition.

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

Notes: For In vitro laboratory use only. Not for any clinical, therapeutic, or diagnostic use in humans or animals. Not for animal or human consumption.