BCKDE1A Antibody (H-5): sc-271538

BCKDE1A Antibody (H-5) is a mouse monoclonal IgG2a kappa light chain antibody that detects BCKDE1A protein of mouse, rat, and human origin by western blotting (WB), immunoprecipitation (IP), immunofluorescence (IF), immunohistochemistry, and enzyme-linked immunosorbent assay (ELISA). BCKDE1A (H-5) antibody 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. BCKDE1A protein, also known as branched-chain α-keto acid dehydrogenase E1 component α chain or BCKDHA, plays a crucial role in the catabolism of branched-chain amino acids by forming part of the inner mitochondrial membrane complex. This complex is essential for converting α-keto acids into acyl-CoA and carbon dioxide, a process vital for energy production and metabolic regulation. BCKDE1A is the α chain of the heterotetrameric BCKDE1 complex, which consists of two α chains and two β chains. Mutations in BCKDE1A can disrupt the assembly of the BCKDH complex, leading to a deficiency that results in the accumulation of branched-chain amino acids in the blood and urine, a condition known as type IA maple syrup urine disease (MSUD). Anti-BCKDE1A antibody (H-5) effectively detects this protein across multiple applications, making BCKDE1A (H-5) monoclonal antibody an invaluable tool for researchers studying metabolic disorders and mitochondrial function.

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BCKDE1A Antibody (H-5) References:

1. Maple syrup urine disease-treatment and outcome in patients of Turkish descent in Germany.  |  Simon, E., et al. 2005. Turk J Pediatr. 47: 8-13. PMID: 15884622
2. alpha-keto acids accumulating in maple syrup urine disease stimulate lipid peroxidation and reduce antioxidant defences in cerebral cortex from young rats.  |  Bridi, R., et al. 2005. Metab Brain Dis. 20: 155-67. PMID: 15938133
3. Evidence that intracellular Ca2+ mediates the effect of alpha-ketoisocaproic acid on the phosphorylating system of cytoskeletal proteins from cerebral cortex of immature rats.  |  Funchal, C., et al. 2005. J Neurol Sci. 238: 75-82. PMID: 16111708
4. Morphological alterations and cell death provoked by the branched-chain alpha-amino acids accumulating in maple syrup urine disease in astrocytes from rat cerebral cortex.  |  Funchal, C., et al. 2005. Cell Mol Neurobiol. 25: 851-67. PMID: 16133938
5. A new protein substitute for adolescents and adults with maple syrup urine disease (MSUD).  |  Hallam, P., et al. 2005. J Inherit Metab Dis. 28: 665-72. PMID: 16151896
6. Branched-chain alpha-keto acids accumulating in maple syrup urine disease induce reorganization of phosphorylated GFAP in C6-glioma cells.  |  Funchal, C., et al. 2005. Metab Brain Dis. 20: 205-17. PMID: 16167198
7. Computational disease gene identification: a concert of methods prioritizes type 2 diabetes and obesity candidate genes.  |  Tiffin, N., et al. 2006. Nucleic Acids Res. 34: 3067-81. PMID: 16757574
8. Mutational spectrum of maple syrup urine disease in Spain.  |  Rodríguez-Pombo, P., et al. 2006. Hum Mutat. 27: 715. PMID: 16786533
9. Molecular basis of maple syrup urine disease: novel mutations at the E1 alpha locus that impair E1(alpha 2 beta 2) assembly or decrease steady-state E1 alpha mRNA levels of branched-chain alpha-keto acid dehydrogenase complex.  |  Chuang, JL., et al. 1994. Am J Hum Genet. 55: 297-304. PMID: 8037208

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-BCKDE1A Antibody (H-5) has 7 citations in a variety of scientific publications.

Primary antibodies like Anti-BCKDE1A Antibody (H-5) 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.

BCKDE1A Antibody (H-5) Specifications

Alternate Product Name: BCKDE1A

Product Clone Name: H-5

Classification: Primary Antibody

Clonality: Monoclonal Antibody

Antibody Isotype: IgG_2a kappa light chain

Amino Acids: 47-346

Epitope Target: BCKDE1A

Epitope Species: human

Product Reactivity/Detection: BCKDE1A

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_2a kappa light chain in 1.0 ml of PBS with < 0.1% sodium azide and 0.1% gelatin

Research Citations: 7 citations in various scientific publications

Available Conjugates/Forms for BCKDE1A Antibody (H-5): 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, X: TransCruz Reagent