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PDH-E1α Antibody (D-6): sc-377092
- PDH-E1α Antibody (D-6) is a mouse monoclonal IgG2a κ PDH-E1α antibody, cited in 70 publications, provided at 200 µg/ml
- raised against amino acids 31-161 mapping near the N-terminus of PDH-E1α of human origin
- PDH-E1 alpha Antibody (D-6) is recommended for detection of PDH-E1α of mouse, rat and human origin by WB, IP, IF, IHC(P) and ELISA; also reactive with additional species, including and bovine and porcine
- Anti-PDH-E1 alpha Antibody (D-6) is available conjugated to agarose for IP; HRP for WB, IHC(P) and ELISA; and to either phycoerythrin or FITC for IF, IHC(P) and FCM
- also available conjugated to Alexa Fluor® 488, Alexa Fluor® 546, Alexa Fluor® 594 or Alexa Fluor® 647 for WB (RGB), IF, IHC(P) and FCM, and for use with RGB fluorescent imaging systems, such as iBright™ FL1000, FluorChem™, Typhoon, Azure and other comparable systems
- also available conjugated to Alexa Fluor® 680 or Alexa Fluor® 790 for WB (NIR), IF and FCM; for use with Near-Infrared (NIR) detection systems, such as LI-COR®Odyssey®, iBright™ FL1000, FluorChem™, Typhoon, Azure and other comparable systems
- Contact our Technical Service Department (or your local Distributor) for more information on how to receive a FREE 10 µg sample of PDH-E1α (D-6): sc-377092.
- m-IgG Fc BP-HRP, m-IgG2a BP-HRP and m-IgGκ BP-HRP are the preferred secondary detection reagents for PDH-E1α Antibody (D-6) for WB and IHC(P) applications. These reagents are now offered in bundles with PDH-E1α Antibody (D-6) (see ordering information below).
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PDH-E1α Antibody (D-6) is an IgG2a κ mouse monoclonal PDH-E1 alpha antibody (also designated ODPA antibody, Pyruvate Dehydrogenase E1 Subunit Alpha 1 antibody, Pyruvate Dehydrogenase E1 Component Subunit Alpha Somatic Form Mitochondrial antibody, Pyruvate Dehydrogenase (Lipoamide) Alpha 1 antibody, Pyruvate Dehydrogenase Alpha 1 antibody, PDHE1-A Type I antibody, PHE1A antibody, PDHCE1A antibody, PDHAD antibody, or PDHA antibody) that detects the PDH-E1 alpha protein of mouse, rat and human origin by WB, IP, IF, IHC(P) and ELISA. PDH-E1α Antibody (D-6) is available as both the non-conjugated anti-PDH-E1 alpha antibody form, as well as multiple conjugated forms of anti-PDH-E1 alpha antibody, including agarose, HRP, PE, FITC and multiple Alexa Fluor® conjugates. The pyruvate dehydrogenase (PDH) complex is a nuclear-encoded mitochondrial matrix enzyme complex that functions as the primary link between glycolysis and the tricarboxylic acid (TCA) cycle by catalyzing the irreversible conversion of pyruvate into acetyl-CoA. The E1 enzyme of the PDH complex is made up of a heterotetramer of two α and two β subunits. The E1-α subunit (PDH-E1α) contains the E1 active site and plays a key role in the function of the PDH complex. The PDH complex is regulated by phosphorylation and dephosphorylation of PDH-E1α. The gene encoding for PDH-E1α maps to chromosome Xp22-q24, and a 20-bp deletion in the last exon of this gene is sufficient to cause PDH deficiency, which causes a broad range of symptoms including the development of seizures, mental retardation and spasticity, as well as intermittent episodes of lactic acidosis associated with cerebellar ataxia.
Alexa Fluor® is a trademark of Molecular Probes Inc., OR., USA
LI-COR® and Odyssey® are registered trademarks of LI-COR Biosciences
References:
- A case of PDH-E1 alpha mosaicism in a male patient with severe metabolic lactic acidosis. | Seyda, A., et al. 2001. J Inherit Metab Dis. 24: 551-9. PMID: 11757583
- PDH-E1alpha dephosphorylation and activation in human skeletal muscle during exercise: effect of intralipid infusion. | Pilegaard, H., et al. 2006. Diabetes. 55: 3020-7. PMID: 17065338
- Pyruvate dehydrogenase deficiency due to a 20-bp deletion in exon II of the pyruvate dehydrogenase (PDH) E1 alpha gene. | Chun, K., et al. 1991. Am J Hum Genet. 49: 414-20. PMID: 1907799
- Characterisation of a cDNA for porcine PDH-E1 alpha and comparison with the human cDNA. | Sermon, K., et al. 1990. Nucleic Acids Res. 18: 4925. PMID: 2395657
- Pyruvate dehydrogenase deficiency caused by a 33 base pair duplication in the PDH E1 alpha subunit. | Hansen, LL., et al. 1994. Hum Mol Genet. 3: 1021-2. PMID: 7545958
- Pyruvate dehydrogenase deficiency. | Brown, GK., et al. 1994. J Med Genet. 31: 875-9. PMID: 7853374
- Mutations in the X-linked E1 alpha subunit of pyruvate dehydrogenase leading to deficiency of the pyruvate dehydrogenase complex. | Chun, K., et al. 1993. Hum Mol Genet. 2: 449-54. PMID: 8504306
- Evolution of mammalian X-linked and autosomal Pgk and Pdh E1 alpha subunit genes. | Fitzgerald, J., et al. 1996. Mol Biol Evol. 13: 1023-31. PMID: 8752009
- Association of cerebral dysgenesis and lactic acidemia with X-linked PDH E1 alpha subunit mutations in females. | Otero, LJ., et al. 1995. Pediatr Neurol. 13: 327-32. PMID: 8771169
Ordering Information
| Product Name | Catalog # | UNIT | Price | Qty | FAVORITES | |
PDH-E1α Antibody (D-6) | sc-377092 | 200 µg/ml | $310.00 | |||
PDH-E1α Antibody (D-6): m-IgG Fc BP-HRP Bundle | sc-529971 | 200 µg Ab; 10 µg BP | $348.00 | |||
PDH-E1α Antibody (D-6): m-IgGκ BP-HRP Bundle | sc-523183 | 200 µg Ab, 40 µg BP | $348.00 | |||
PDH-E1α Antibody (D-6): m-IgG2a BP-HRP Bundle | sc-547409 | 200 µg Ab; 10 µg BP | $348.00 | |||
PDH-E1α Antibody (D-6) AC | sc-377092 AC | 500 µg/ml, 25% agarose | $408.00 | |||
PDH-E1α Antibody (D-6) HRP | sc-377092 HRP | 200 µg/ml | $310.00 | |||
PDH-E1α Antibody (D-6) FITC | sc-377092 FITC | 200 µg/ml | $324.00 | |||
PDH-E1α Antibody (D-6) PE | sc-377092 PE | 200 µg/ml | $337.00 | |||
PDH-E1α Antibody (D-6) Alexa Fluor® 488 | sc-377092 AF488 | 200 µg/ml | $350.00 | |||
PDH-E1α Antibody (D-6) Alexa Fluor® 546 | sc-377092 AF546 | 200 µg/ml | $350.00 | |||
PDH-E1α Antibody (D-6) Alexa Fluor® 594 | sc-377092 AF594 | 200 µg/ml | $350.00 | |||
PDH-E1α Antibody (D-6) Alexa Fluor® 647 | sc-377092 AF647 | 200 µg/ml | $350.00 | |||
PDH-E1α Antibody (D-6) Alexa Fluor® 680 | sc-377092 AF680 | 200 µg/ml | $350.00 | |||
PDH-E1α Antibody (D-6) Alexa Fluor® 790 | sc-377092 AF790 | 200 µg/ml | $350.00 |