Pyruvate dehydrogenase (PDH) subunit E2/E3bp monoclonal antibody
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Catalog No. MSP06
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$325.00 - 100 µg
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This antibody can also be purchased as part of a Sample Pack.
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UniProt Number:
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Subunit (E2): P10515
Subunit (E3bp): O00330
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Alternate Names:
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Dihydrolipoyllysine-residue acetyltransferase component of pyruvate dehydrogenase complex, mitochondrial, Pyruvate dehydrogenase (Lipoamide) alpha 1, 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) M2 antigen complex 70 kDa subunit
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Structure and Function:
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The pyruvate dehydrogenase complex contains three different subunits including the dihydrolipoamide acetyltransferase (E2) subunit as well as the lipoamide dehydrogenase (E3) subunit.
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Disease Associations:
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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.
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Product Specifications
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Applications:
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Western blotting, Immunohistochemistry, and Immunocytochemistry (heat-induced antigen-retrieval improves signal)
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Species Reactivity:
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human (two bands), bovine, rat and mouse
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Host Species:
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mouse
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Isotype:
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IgG2a, k
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Clone ID:
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13G2AE2BH5
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Concentration:
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1.0 mg/mL in Hepes-Buffered Saline (HBS) with 0.02% azide as a preservative.
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Suggested Working Concentration:
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1 µg/mL for Western blotting
1 µg/mL for Immunocytochemistry
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Storage Conditions:
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4°C
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Country of Origin:
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USA
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WB Images
(click to enlarge)
Figure 1. Isolated mitochondria from human heart (lane 1), bovine heart (lane 2), rat heart (lane 3), mouse heart (lane 4), and HepG2 (lane 5) detected with (MSP06) anti-PDH-E2/E3bp antibody.
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ICC Images
(click to enlarge)
Figure 2. Mitochondrial localization of PDH in cultured, normal human embryonic lung fibroblasts (strain MRC5) labeled with anti-PDH-E2/E3bp mAb 13G2AE2BH5 (MSP06) as the primary antibody (1 ug/ml) and an AlexaFluor® 488 goat anti-mouse IgG2a secondary antibody (2 ug/ml).
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IHC Images
(click to enlarge)
Figure 3. PDH subunit E2/E3bp immunohistochemistry in human cerebellum visualized with mAb MSP06. PDH subunit E2/E3bp immunoactivity is most intense in neuronal cell bodies, most notably in the large Purkinje cells.
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