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Branched Chain Keto Acid Dehydrogenase E1, alpha Polypeptide Proteins (BCKDHA)

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 Antibodies (21) and many more products for this protein.

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

Showing 9 out of 10 products:

Catalog No. Origin Source Conjugate Images Quantity Supplier Delivery Price Details
HOST_Escherichia coli (E. coli) Human His tag „Crystallography Grade“ protein due to multi-step, protein-specific purification process 1 mg Log in to see 29 to 34 Days
$4,331.68
Details
HOST_Escherichia coli (E. coli) Mouse His tag „Crystallography Grade“ protein due to multi-step, protein-specific purification process 1 mg Log in to see 29 to 34 Days
$4,331.68
Details
HOST_Escherichia coli (E. coli) Human His tag 10 μg Log in to see 7 to 8 Days
$319.00
Details
HOST_Wheat germ Human GST tag 10 μg Log in to see 9 Days
$405.71
Details
Yeast Macaca fascicularis His tag   1 mg Log in to see 56 to 66 Days
$3,107.50
Details
Yeast Pan troglodytes His tag   1 mg Log in to see 56 to 66 Days
$3,107.50
Details
Yeast Rat His tag   1 mg Log in to see 56 to 66 Days
$3,107.50
Details
Yeast Cow His tag   1 mg Log in to see 56 to 66 Days
$3,107.50
Details
HOST_Escherichia coli (E. coli) Human Un-conjugated   10 μg Log in to see 3 to 4 Days
$364.64
Details

BCKDHA Proteins by Origin and Source

Origin Expressed in Conjugate
Human ,
,
Mouse (Murine)

Rat (Rattus)

Top referenced BCKDHA Proteins

  1. Human BCKDHA Protein expressed in Escherichia coli (E. coli) - ABIN667373 : Nobukuni, Mitsubuchi, Hayashida, Ohta, Indo, Ichiba, Endo, Matsuda et al.: Heterogeneity of mutations in maple syrup urine disease (MSUD): screening and identification of affected E1 alpha and E1 beta subunits of the branched-chain alpha-keto-acid dehydrogenase multienzyme ... in Biochimica et biophysica acta 1993 (PubMed)
    Show all 2 references for ABIN667373

More Proteins for Branched Chain Keto Acid Dehydrogenase E1, alpha Polypeptide (BCKDHA) Interaction Partners

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

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

  2. The novel DBT (show DBT Proteins) 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.

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

  4. 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]

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

  6. A list of nine primary candidate genes for T2D and five for obesity were identified in this paper. Two genes, LPL (show LCP1 Proteins) and BCKDHA, were common to these two sets.

  7. BCKDHA and BCKDHB (show BCKDHB Proteins) mutations might be primarily responsible for maple syrup urine disease in the Indian population.

  8. autism presenting with intellectual disability and epilepsy caused by BCKDK (show BCKDK Proteins) mutations represents a potentially treatable syndrome.

  9. 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

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

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

  1. analysis of NCF2 (show NCF2 Proteins), BCKDHB (show BCKDHB Proteins) and BCKDHA in pig

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

  1. Disruption of the BDK (show KNG1 Proteins) gene establishes that regulation of BCKDH by phosphorylation is critically important for the regulation of oxidative disposal of branched-chain amino acids.

BCKDHA Protein 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

  • branched chain keto acid dehydrogenase E1, alpha polypeptide (BCKDHA)
  • branched chain keto acid dehydrogenase E1, alpha polypeptide (bckdha)
  • branched chain keto acid dehydrogenase E1, alpha polypeptide (LOC100352048)
  • 2-oxoisovalerate dehydrogenase alpha subunit (branched-chain alpha-keto acid dehydrogenase E1 component alpha chain) (BCKDH E1-alpha) (BCKDE1A) (BckdhA)
  • branched chain ketoacid dehydrogenase E1, alpha polypeptide (Bckdha)
  • BCKDA protein
  • BCKDE1A protein
  • E1a protein
  • MSU protein
  • MSUD1 protein
  • OVD1A protein
  • wu:fd20d04 protein
  • zgc:110049 protein

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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