anti-Branched Chain Keto Acid Dehydrogenase E1, alpha Polypeptide (BCKDHA) Antibodies

The branched-chain alpha-keto acid (BCAA) dehydrogenase (BCKD) complex is an innter mitochondrial enzyme complex that catalyzes the second major step in the catabolism of the branched-chain amino acids leucine, isoleucine, and valine. Additionally we are shipping BCKDHA Proteins (11) and BCKDHA Kits (9) and many more products for this protein.

list all antibodies Gene Name GeneID UniProt
BCKDHA 593 P12694
BCKDHA 12039  
BCKDHA 25244 P11960
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Top anti-BCKDHA Antibodies at antibodies-online.com

Showing 10 out of 29 products:

Catalog No. Reactivity Host Conjugate Application Images Quantity Supplier Delivery Price Details
Cow Rabbit Un-conjugated IHC, WB WB Suggested Anti-BCKDHA Antibody Titration: 0.2-1 ug/mlELISA Titer: 1:312500Positive Control: MCF7 cell lysate 100 μL Log in to see 2 to 3 Days
$319.00
Details
Human Rabbit Un-conjugated EIA, WB 0.4 mL Log in to see 6 to 8 Days
$484.00
Details
Human Rabbit Un-conjugated FACS, IHC (p), WB Western blot analysis of BCKDHA Antibody (C-term) (ABIN390811) in Jurkat cell line lysates (35 µg/lane). BCKDHA (arrow) was detected using the purified polyclonal antibody. Formalin-fixed and paraffin-embedded human lung carcinoma reacted with BCKDHA Antibody (C-term), which was peroxidase-conjugated to the secondary antibody, followed by DAB staining. 400 μL Log in to see 10 to 11 Days
$385.00
Details
Human Rabbit Un-conjugated ICC, IF, IHC, IHC (p), WB Human Kidney: Formalin-Fixed, Paraffin-Embedded (FFPE) 50 μL Log in to see 11 to 14 Days
$484.00
Details
Cow Rabbit Un-conjugated WB 50 μg Log in to see 11 to 14 Days
$551.83
Details
Human Rabbit Un-conjugated WB Western blot analysis of extracts of various cells, using BCKDHA antibody. 100 μL Log in to see 16 Days
$366.77
Details
Human Rabbit Un-conjugated ELISA, WB 50 μg Log in to see 2 to 3 Days
$446.88
Details
Human Rabbit Un-conjugated WB BCKDHA antibody used at 1 ug/ml to detect target protein. 50 μg Log in to see 9 to 11 Days
$510.36
Details
Human Mouse Un-conjugated ELISA, WB BCKDHA polyclonal antibody (A01), Lot # 051212JC01 Western Blot analysis of BCKDHA expression in Y-79 . Western Blot detection against Immunogen (37 KDa) . 50 μL Log in to see 11 to 12 Days
$227.50
Details
Human Mouse Un-conjugated WB Western Blot analysis of BCKDHA expression in transfected 293T cell line by BCKDHA MaxPab polyclonal antibody.Lane 1: BCKDHA transfected lysate(48.95 KDa).Lane 2: Non-transfected lysate. 50 μg Log in to see 11 to 12 Days
$348.04
Details

More Antibodies against BCKDHA Interaction Partners

Human Branched Chain Keto Acid Dehydrogenase E1, alpha Polypeptide (BCKDHA) interaction partners

  1. pathogenic effect of a novel variant of BCKDHA leading to classical maple syrup urine disease

  2. Data suggest that the following genetic modifications are involved in patients with maple syrup urine disease in Iran: (1) mutation in BCKDHA (branched chain keto acid dehydrogenase E1 alpha); (2) mutation in BCKDHB (branched chain keto acid dehydrogenase E1 subunit beta); (3) mutation in DBT (dihydrolipoamide branched chain transacylase E2; one patient).

  3. we identified seven novel genetic variants: p.Leu549Pro, p.Glu564*, p.Leu641Pro in MUT, p.Tyr206Cys in PCCB, p.His194Arg, p.Val298Met in BCKDHA and p.Glu286_Met290del in BCKDHB gene. In silico and/or eukaryotic expression studies confirmed pathogenic effect of all novel genetic variants

  4. Five novel mutations in BCKDHA were identified in MSUD patients.

  5. The novel DBT mutation c.650-651insT was more prevalent than the deleted 4.7-kb heterozygote in the Amis population. The reported 4.7-kb deletion indicating a possible founder mutation may be preserved.

  6. Targeted parallel sequencing revealed novel mutations in the gene BCKDHA for prenatal testing of maple syrup urine disease.

  7. Data from infant/her heterozygous parents (first cousins) suggest homozygous mutation (S144I) in BCKDHA can result in maple syrup urine disease (IA); molecular modeling suggests this missense mutation in exon 4 affects protein stability. [CASE STUDY]

  8. Case Report: functional characterization of the novel intronic nucleotide change c.288+9C>T within the BCKDHA gene in maple syrup urine disease.

  9. A list of nine primary candidate genes for T2D and five for obesity were identified in this paper. Two genes, LPL and BCKDHA, were common to these two sets.

  10. BCKDHA and BCKDHB mutations might be primarily responsible for maple syrup urine disease in the Indian population.

  11. autism presenting with intellectual disability and epilepsy caused by BCKDK mutations represents a potentially treatable syndrome.

  12. identified 4 novel mutations of the BCKDHA gene in 3 Korean newborns; to the best of knowledge, this is the first report of maple syrup urine disease confirmed by genetic analysis in Korea

  13. Case Report: Maple syrup urine disease due to a new large deletion at BCKDHA caused by non-homologous recombination.

  14. five mutations, three of them novel, responsible for maple syrup urine disease

  15. the conformational stability underlying the folding of this lipoic acid bearing domain of human mitochondrial branched chain alpha-ketoacid dehydrogenase

  16. in our cohort more severe enzyme & clinical phenotypes of variant maple syrup urine disease were mainly associated with specific genotypes in BCKDHA gene; milder enzyme & clinical phenotypes were associated with specific genotypes in BCKDHB & DBT genes

  17. 30 Maple syrup urine disease Portuguese patients studied; 17 putative mutations have been identified (6 in BCKDHA, 5 in BCKDHB and 6 in DBT); 7 of are described for the first time.

  18. A founder mutation in the BCKDHA is responsible for the high incidence of the maple syrup urine disease among Portuguese Gypsies.

  19. In 37% (12 patients) of a total of 64 alleles, the supposed maple syrup urine disease-causing mutations in Turkish patients were located in the BCKDHA gene, in 44% (14 patients) in the BCKDHB gene and in 19% (6 patients) in the DBT gene.

Pig (Porcine) Branched Chain Keto Acid Dehydrogenase E1, alpha Polypeptide (BCKDHA) interaction partners

  1. analysis of NCF2, BCKDHB and BCKDHA in pig

Mouse (Murine) Branched Chain Keto Acid Dehydrogenase E1, alpha Polypeptide (BCKDHA) interaction partners

  1. Disruption of the BDK gene establishes that regulation of BCKDH by phosphorylation is critically important for the regulation of oxidative disposal of branched-chain amino acids.

BCKDHA Antigen Profile

Protein Summary

The branched-chain alpha-keto acid (BCAA) dehydrogenase (BCKD) complex is an innter mitochondrial enzyme complex that catalyzes the second major step in the catabolism of the branched-chain amino acids leucine, isoleucine, and valine. The BCKD complex consists of three catalytic components: a heterotetrameric (alpha2-beta2) branched-chain alpha-keto acid decarboxylase (E1), a dihydrolipoyl transacylase (E2), and a dihydrolipoamide dehydrogenase (E3). This gene encodes the alpha subunit of the decarboxylase (E1) component. Mutations in this gene result in maple syrup urine disease, type IA. Multiple transcript variants encoding different isoforms have been found for this gene.

Gene names and symbols associated with BCKDHA

  • 2-oxoisovalerate dehydrogenase subunit alpha, mitochondrial (LOC704978) antibody
  • branched chain keto acid dehydrogenase E1, alpha polypeptide (bckdha) antibody
  • branched chain keto acid dehydrogenase E1, alpha polypeptide (BCKDHA) antibody
  • 2-oxoisovalerate dehydrogenase alpha subunit (branched-chain alpha-keto acid dehydrogenase E1 component alpha chain) (BCKDH E1-alpha) (BCKDE1A) (BckdhA) antibody
  • branched chain ketoacid dehydrogenase E1, alpha polypeptide (Bckdha) antibody
  • BCKDA antibody
  • BCKDE1A antibody
  • E1a antibody
  • MSU antibody
  • MSUD1 antibody
  • OVD1A antibody
  • wu:fd20d04 antibody
  • zgc:110049 antibody

Protein level used designations for BCKDHA

branched chain keto acid dehydrogenase E1, alpha polypeptide , 2-oxoisovalerate dehydrogenase subunit alpha, mitochondrial , 2-oxoisovalerate dehydrogenase alpha subunit (branched-chain alpha-keto acid dehydrogenase E1 component alpha chain) (BCKDH E1-alpha) (BCKDE1A) , 2-oxoisovalerate dehydrogenase (lipoamide) , BCKDH E1-alpha , branched-chain alpha-keto acid dehydrogenase E1 component alpha chain , branched chain keto acid dehydrogenase E1 alpha polypeptide , BCKDE1A , branched chain keto acid dehydrogenase E1, alpha polypeptide (maple syrup urine disease) , BCKAD E1[a] , branched chain alpha-ketoacid dehydrogenase subunit E1 alpha , branched chain keto acid dehydrogenase subunit E1, alpha polypeptide

GENE ID SPECIES
704978 Macaca mulatta
100036656 Xenopus (Silurana) tropicalis
100068678 Equus caballus
100145857 Ovis aries
100352048 Oryctolagus cuniculus
374210 Gallus gallus
3708743 Pseudoalteromonas haloplanktis TAC125
554124 Danio rerio
593 Homo sapiens
484488 Canis lupus familiaris
100142666 Sus scrofa
282149 Bos taurus
468889 Pan troglodytes
12039 Mus musculus
25244 Rattus norvegicus
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