Branched Chain Keto Acid Dehydrogenase E1, beta Polypeptide Proteins (BCKDHB)

Branched-chain keto acid dehydrogenase is a multienzyme complex associated with the inner membrane of mitochondria, and functions in the catabolism of branched-chain amino acids. Additionally we are shipping BCKDHB Antibodies (34) and and many more products for this protein.

list all proteins Gene Name GeneID UniProt
BCKDHB 29711 P35738
BCKDHB 594 P21953
BCKDHB 12040 Q6P3A8
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Top BCKDHB Proteins at antibodies-online.com

Showing 6 out of 6 products:

Catalog No. Origin Source Conjugate Images Quantity Supplier Delivery Price Details
Insect Cells Human His tag „Crystallography Grade“ protein due to multi-step, protein-specific purification process 1 mg Log in to see 50 Days
$6,869.04
Details
Insect Cells Mouse His tag „Crystallography Grade“ protein due to multi-step, protein-specific purification process 1 mg Log in to see 50 Days
$6,869.04
Details
HEK-293 Cells Human Myc-DYKDDDDK Tag Validation with Western Blot 20 μg Log in to see Available
$814.00
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Wheat germ Human GST tag 2 μg Log in to see 11 to 12 Days
$225.40
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Yeast Rat His tag   1 mg Log in to see 60 to 71 Days
$2,927.83
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Yeast Cow His tag   1 mg Log in to see 60 to 71 Days
$2,927.83
Details

BCKDHB Proteins by Origin and Source

Origin Expressed in Conjugate
Rat (Rattus)

Human , ,
, ,
Mouse (Murine)

More Proteins for Branched Chain Keto Acid Dehydrogenase E1, beta Polypeptide (BCKDHB) Interaction Partners

Pig (Porcine) Branched Chain Keto Acid Dehydrogenase E1, beta Polypeptide (BCKDHB) interaction partners

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

Human Branched Chain Keto Acid Dehydrogenase E1, beta Polypeptide (BCKDHB) interaction partners

  1. Data suggest that the following genetic modifications are involved in patients with maple syrup urine disease in Iran: (1) mutation in BCKDHA (show BCKDHA Proteins) (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 (show DBT Proteins); one patient).

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

  3. Five novel mutations in BCKDHB were identified in MSUD patients.

  4. we describe the presenting symptoms and clinical course of a Chinese boy with intermittent Intermittent maple syrup urine disease from BCKDHB gene mutation

  5. Mutations in BCKDHB gene is associated with maple syrup urine disease.

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

  7. A key tyrosine residue in the E1b active site functions as a conformational switch to reduce the reactivity of the thiamine diphosphate cofactor through interactions with its thiazolium ring.

  8. the two active sites in the E1b heterotetramer operate independently during the ThDP-dependent decarboxylation reaction

  9. Reconstructed haplotype from BCKDHB variants was associated with premature ovarian failure.

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

Mouse (Murine) Branched Chain Keto Acid Dehydrogenase E1, beta Polypeptide (BCKDHB) 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.

BCKDHB Protein Profile

Protein Summary

Branched-chain keto acid dehydrogenase is a multienzyme complex associated with the inner membrane of mitochondria, and functions in the catabolism of branched-chain amino acids. The complex consists of multiple copies of 3 components: branched-chain alpha-keto acid decarboxylase (E1), lipoamide acyltransferase (E2) and lipoamide dehydrogenase (E3). This gene encodes the E1 beta subunit, and mutations therein have been associated with maple syrup urine disease (MSUD), type 1B, a disease characterized by a maple syrup odor to the urine in addition to mental and physical retardation, and feeding problems. Alternative splicing at this locus results in transcript variants with different 3' non-coding regions, but encoding the same isoform.

Gene names and symbols associated with BCKDHB

  • branched chain keto acid dehydrogenase E1, beta polypeptide (BCKDHB)
  • branched chain keto acid dehydrogenase E1 subunit beta (BCKDHB)
  • branched chain keto acid dehydrogenase E1, beta polypeptide (bckdhb)
  • branched chain keto acid dehydrogenase E1, beta polypeptide S homeolog (bckdhb.S)
  • branched chain keto acid dehydrogenase E1 subunit beta (Bckdhb)
  • branched chain ketoacid dehydrogenase E1, beta polypeptide (Bckdhb)
  • BCKDHB protein
  • dJ279A18.1 protein
  • E1B protein
  • zgc:158777 protein

Protein level used designations for BCKDHB

2-oxoisovalerate dehydrogenase subunit beta, mitochondrial , branched chain keto acid dehydrogenase E1, beta polypeptide (maple syrup urine disease) , mitochondrial branched-chain alpha-keto acid dehydrogenase E1-beta subunit , branched chain keto acid dehydrogenase E1, beta polypeptide , branched chain keto acid dehydrogenase E1 beta polypeptide , branched chain keto acid dehydrogenase E1 beta-like , 2-oxoisovalerate dehydrogenase beta subunit , BCKDE1B , BCKDH E1-beta , branched chain keto acid dehydrogenase E1 beta , branched-chain alpha-keto acid dehydrogenase E1 component beta chain , E1b-beta subunit of the branched-chain complex , branched chain alpha-ketoacid dehydrogenase E1-beta subunit

GENE ID SPECIES
395375 Gallus gallus
462845 Pan troglodytes
474978 Canis lupus familiaris
569980 Danio rerio
100023799 Monodelphis domestica
100065300 Equus caballus
100137712 Xenopus laevis
100142669 Sus scrofa
100173401 Pongo abelii
100353682 Oryctolagus cuniculus
100597493 Nomascus leucogenys
29711 Rattus norvegicus
594 Homo sapiens
282150 Bos taurus
12040 Mus musculus
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