BCKDHB Antibody (H-6): sc-374630

BCKDHB Antibody (H-6) is a mouse monoclonal IgG2b kappa light chain antibody that detects BCKDHB protein of mouse, rat, and human origin by western blotting (WB), immunoprecipitation (IP), immunofluorescence (IF), and enzyme-linked immunosorbent assay (ELISA). Anti-BCKDHB 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. BCKDHB protein, also known as branched-chain keto acid dehydrogenase E1, β polypeptide, is a crucial 392 amino acid component of the branched-chain keto acid dehydrogenase complex, which plays a vital role in the catabolism of branched-chain amino acids such as leucine, isoleucine, and valine. BCKDHB is located in the mitochondrial matrix, where BCKDHB facilitates the conversion of these amino acids into energy, underscoring BCKDHB′s importance in metabolic processes. Deficiencies in BCKDHB can lead to serious health issues, including maple syrup urine disease type IB (MSUD1B), a genetic disorder characterized by the accumulation of toxic metabolites, resulting in neurological damage and severe developmental challenges. The gene encoding BCKDHB is located on human chromosome 6q14.1, and understanding BCKDHB function and interactions is essential for developing therapeutic strategies for related metabolic disorders.

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BCKDHB Antibody (H-6) References:

1. Structural and biochemical basis for novel mutations in homozygous Israeli maple syrup urine disease patients: a proposed mechanism for the thiamin-responsive phenotype.  |  Chuang, JL., et al. 2004. J Biol Chem. 279: 17792-800. PMID: 14742428
2. A versatile conformational switch regulates reactivity in human branched-chain alpha-ketoacid dehydrogenase.  |  Machius, M., et al. 2006. Structure. 14: 287-98. PMID: 16472748
3. Branched chain alpha-keto acid dehydrogenase, E1-beta subunit gene is associated with premature ovarian failure.  |  Kang, H., et al. 2008. Fertil Steril. 89: 728-31. PMID: 17524396
4. Regional assignment of two genes of the human branched-chain alpha-keto acid dehydrogenase complex: the E1 beta gene (BCKDHB) to chromosome 6p21-22 and the E2 gene (DBT) to chromosome 1p31.  |  Zneimer, SM., et al. 1991. Genomics. 10: 740-7. PMID: 1889817
5. Molecular cloning of the mature E1b-beta subunit of human branched-chain alpha-keto acid dehydrogenase complex.  |  Chuang, JL., et al. 1990. FEBS Lett. 262: 305-9. PMID: 2335211
6. Mammalian alpha-keto acid dehydrogenase complexes: gene regulation and genetic defects.  |  Patel, MS. and Harris, RA. 1995. FASEB J. 9: 1164-72. PMID: 7672509
7. Maple syrup urine disease: the E1beta gene of human branched-chain alpha-ketoacid dehydrogenase complex has 11 rather than 10 exons, and the 3' UTR in one of the two E1beta mRNAs arises from intronic sequences.  |  Chuang, JL., et al. 1996. Am J Hum Genet. 58: 1373-7. PMID: 8651316
8. Maple syrup urine disease: it has come a long way.  |  Chuang, DT. 1998. J Pediatr. 132: S17-23. PMID: 9546032

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-BCKDHB Antibody (H-6) has 3 citations in a variety of scientific publications.

Primary antibodies like Anti-BCKDHB 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.

BCKDHB Antibody (H-6) Specifications

Alternate Product Name: BCKDHB

Product Clone Name: H-6

Classification: Primary Antibody

Clonality: Monoclonal Antibody

Antibody Isotype: IgG_2b kappa light chain

Amino Acids: 67-97

Epitope Target: BCKDHB

Epitope Species: human

Product Reactivity/Detection: BCKDHB

Species Reactivity/Detection: mouse, rat, human

Additional Mammalian Reactivity: N/A

Additional Non-Mammalian Reactivity: N/A

Applications: WB, IP, IF, 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 BCKDHB 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, P: Blocking Peptide, PE: Phycoerythrin