E3BP Antibody (H-6): sc-377255

E3BP Antibody (H-6) is a mouse monoclonal IgG2b kappa light chain antibody that detects E3BP protein of mouse, rat, and human origin by western blotting (WB), immunoprecipitation (IP), immunofluorescence (IF), immunohistochemistry, and enzyme-linked immunosorbent assay (ELISA). Anti-E3BP antibody (H-6) is available in both non-conjugated and various conjugated forms, including agarose, horseradish peroxidase (HRP), phycoerythrin (PE), fluorescein isothiocyanate (FITC), and multiple Alexa Fluor® conjugates. E3BP protein, also known as PDHX (Pyruvate dehydrogenase protein X component), plays a crucial role in the pyruvate dehydrogenase (PDH) complex, which is essential for cellular metabolism as this complex serves as the primary link between glycolysis and the tricarboxylic acid (TCA) cycle by catalyzing the conversion of pyruvate into acetyl-CoA. E3BP is a 501 amino acid mitochondrial protein that is vital for anchoring the E3 component to the E2 core of the PDH complex, a process necessary for the complex′s functionality. Deficiencies in E3BP can lead to severe metabolic disorders, including lactic acidosis and developmental delays, highlighting the importance of E3BP in maintaining metabolic homeostasis. Clinical implications of E3BP deficiency can manifest as a range of neurological symptoms, including seizures and cerebellar ataxia, underscoring E3BP′s critical role in both energy metabolism and neurological health.

Alexa Fluor^® is a trademark of Molecular Probes Inc., OR., USA

LI-COR^® and Odyssey^® are registered trademarks of LI-COR Biosciences

E3BP Antibody (H-6) References:

1. Mitochondrial dysfunction in a patient with Joubert syndrome.  |  Morava, E., et al. 2005. Neuropediatrics. 36: 214-7. PMID: 15944909
2. Leigh's disease due to a new mutation in the PDHX gene.  |  Schiff, M., et al. 2006. Ann Neurol. 59: 709-14. PMID: 16566017
3. PDC-E3BP is not a dominant T-cell autoantigen in primary biliary cirrhosis.  |  McHugh, A., et al. 2006. Liver Int. 26: 406-13. PMID: 16629643
4. A new level of architectural complexity in the human pyruvate dehydrogenase complex.  |  Smolle, M., et al. 2006. J Biol Chem. 281: 19772-80. PMID: 16679318
5. A novel gross deletion caused by non-homologous recombination of the PDHX gene in a patient with pyruvate dehydrogenase deficiency.  |  Miné, M., et al. 2006. Mol Genet Metab. 89: 106-10. PMID: 16843025
6. Pyruvate dehydrogenase E3 binding protein (protein X) deficiency.  |  Brown, RM., et al. 2006. Dev Med Child Neurol. 48: 756-60. PMID: 16904023
7. Subunit and catalytic component stoichiometries of an in vitro reconstituted human pyruvate dehydrogenase complex.  |  Brautigam, CA., et al. 2009. J Biol Chem. 284: 13086-98. PMID: 19240034
8. Defects in the E2 lipoyl transacetylase and the X-lipoyl containing component of the pyruvate dehydrogenase complex in patients with lactic acidemia.  |  Robinson, BH., et al. 1990. J Clin Invest. 85: 1821-4. PMID: 2112155

Monoclonal antibodies for mammalian and non-mammalian protein targets represent essentially all targets covered by polyclonal antibodies. Over 8,300 of our antibodies are offered as ImmunoCruz^® conjugates which eliminates the need for a secondary antibody.

Santa Cruz Biotechnology antibodies have over 360,000 research citations. Anti-E3BP Antibody (H-6) has 3 citations in a variety of scientific publications.

Primary antibodies like Anti-E3BP Antibody (H-6) for mammalian target proteins are recommended for the detection of a range of mammalian species, primarily of mouse, rat and human species. Many mammalian antibodies are also ideal for veterinary research and reactive with ovine, porcine, caprine, feline, canine, bovine and equine protein targets. Santa Cruz Biotechnology primary monoclonal antibodies are suitable for laboratory research applications and are not intended for diagnostic or therapeutic use.

E3BP Antibody (H-6) Specifications

Alternate Product Name: E3BP

Product Clone Name: H-6

Classification: Primary Antibody

Clonality: Monoclonal Antibody

Antibody Isotype: IgG_2b kappa light chain

Amino Acids: 151-280

Epitope Target: E3BP

Epitope Species: human

Product Reactivity/Detection: E3BP

Species Reactivity/Detection: mouse, rat, human

Additional Mammalian Reactivity: N/A

Additional Non-Mammalian Reactivity: N/A

Applications: WB, IP, IF, IHC(P), ELISA

Vialing Information: Each vial contains 200 µg IgG_2b kappa light chain in 1.0 ml of PBS with < 0.1% sodium azide and 0.1% gelatin

Research Citations: 3 citations in various scientific publications

Available Conjugates/Forms for E3BP Antibody (H-6): AC: Agarose, AF488: Alexa Fluor^® 488, AF546: Alexa Fluor^® 546, AF594: Alexa Fluor^® 594, AF647: Alexa Fluor^® 647, AF680: Alexa Fluor^® 680, AF790: Alexa Fluor^® 790, FITC: Fluoroscein, HRP: Horseradish Peroxidase, PE: Phycoerythrin