Pyruvate dehydrogenase (PDH) subunit E1 beta monoclonal antibody
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Catalog No. MSP04
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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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P11177
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Alternate Names:
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Pyruvate dehydrogenase E1 component subunit beta, mitochondrial (Short name=PDHE1-B)
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Structure and Function:
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The pyruvate dehydrogenase complex contains three different subunits including the pyruvate dehydrogenase E1 subunit. The PDH complex is responsible for catalyzing the formation of acetyl-CoA and CO2 from pyruvate.
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Disease Associations:
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Enzyme deficiency of the PDH E1 subunit of the PDH complex commonly results in lactic acidosis. There are a wide range of symptoms that characterize this disorder including neonatal death, developmental delay, seizures, ataxia, apnea and X-link Leigh Syndrome (LS). This syndrome presents itself with necrotic lesions that are located symmetrically in both sides the subcortical brain.
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Product Specifications
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Applications:
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Western blotting
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Species Reactivity:
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human, bovine, rat and mouse
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Host Species:
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mouse
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Isotype:
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IgG1, k
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Clone ID:
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17A5E2H8
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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
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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 (MSP04) anti-subunit E1 beta antibody.
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