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Images:

Figure 1. PDH subunit E2 Western blotting image.


Figure 2. PDH subunit E2 Immunocytochemistry image.



PDH subunit E2 monoclonal antibody

Catalog No. MSP05

$325.00 - 100 µg

UniProt Number: P10515
Alternate Names: Dihydrolipoyllysine-residue acetyltransferase component of pyruvate dehydrogenase complex, mitochondrial, M2 antigen complex 70 kDa subunit, Pyruvate dehydrogenase complex E2 subunit (Short name=PDC-E2), (Short name=PDCE2), (Short name=E2) Dihydrolipoamide S-acetyltransferase component of pyruvate dehydrogenase complex 70 kDa mitochondrial autoantigen of primary biliary cirrhosis (Short name=PBC)
Structure and Function: The pyruvate dehydrogenase complex contains three different subunits including the dihydrolipoamide acetyltransferase (E2) subunit.
Disease Associations: The E2 subunit of the PHD complex is associated with primary biliary cirrhosis in that patients diagnosed with this disease have autoantibodies for this subunit. This condition causes damage to liver cells and can result in cirrhosis of the liver. Lactic acidemia (PDH deficiency) is another condition associated with the E2 subunit. The clinical characterization of this disorder includes primary lactic acidosis as well as neurological problems, mainly episodic dystonia.

Product Specifications
 
Applications: Western blotting and Immunocytochemistry (heat-induced antigen-retrieval improves signal)
Species Reactivity: human, bovine
Host Species: mouse
Isotype: IgG1, k
Clone ID: 15D3G9C11
Immunogen: Porcine PDH
Concentration: 1 mg/mL in Phosphate-Buffered Saline (PBS) with 0.02% azide as a preservative.
Suggested Working Concentration: 0.5 µg/mL for Western blotting, 0.2-0.5 µg/mL for Immunocytochemistry
Storage Conditions: 4°C
Country of Origin: USA
Citations: Feng et al., 2009. Down-regulation of mitochondrial acyl carrier protein in mammalian cells compromises protein lipoylation and respiratory complex I and results in cell death.

Ridout et al., 2008. Somatic mosaicism for a PDHA1 mutation in a female with pyruvate dehydrogenase deficiency.

Han et al., 2008. A combined therapeutic approach for pyruvate dehydrogenase deficiency using self-complementary adeno-associated virus serotype-specific vectors and dichloroacetate.

Stiburek et al., 2007. Knockdown of human Oxa1l impairs the biogenesis of F1Fo-ATP synthase and NADH:ubiquinone oxidoreductase.

Koukourakis et al., 2005. Pyruvate dehydrogenase and pyruvate dehydrogenase kinase expression in non small cell lung cancer and tumor-associated stroma.


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