anti-Branched Chain Keto Acid Dehydrogenase E1, beta Polypeptide (BCKDHB) Antibodies

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 Proteins (6) and many more products for this protein.

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

Showing 10 out of 29 products:

Catalog No. Reactivity Host Conjugate Application Images Quantity Supplier Delivery Price Details
Human Rabbit Un-conjugated EIA, FACS, IF, IHC (p), WB   0.4 mL Log in to see 12 to 15 Days
$375.38
Details
Human Rabbit Un-conjugated FACS, IF, IHC (p), WB BCKDHB Antibody (N-term)  western blot analysis in Hela cell line lysates (35ug/lane).This demonstrates the BCKDHB antibody detected the BCKDHB protein (arrow). BCKDHB Antibody (N-term)  immunohistochemistry analysis in formalin fixed and paraffin embedded human hepatocarcinoma followed by peroxidase conjugation of the secondary antibody and DAB staining.  This data demonstrates the use of the BCKDHB Antibody (N-term) for immunohistochemistry.  Clinical relevance has not been evaluated. 400 μL Log in to see 10 to 11 Days
$324.50
Details
Cow Rabbit Un-conjugated WB 100 μL Log in to see 2 to 3 Days
$289.00
Details
Human Rabbit Un-conjugated FACS, ICC, IF, IHC (p), WB Western Blot: BCKDHB Antibody [NBP2-29984] - Flow cytometric analysis of Hela cells (right histogram) compared to a negative control cell (left histogram).FITC-conjugated goat-anti-rabbit secondary antibodies were used for the analysis. Western Blot: BCKDHB Antibody [NBP2-29984] - Western blot analysis in Hela cell line lysates (35ug/lane).This demonstrates the BCKDHB antibody detected the BCKDHB protein (arrow). 0.4 mL Log in to see 10 to 13 Days
$408.19
Details
Human Rabbit Un-conjugated IHC, IHC (p), WB Western Blot: BCKDHB Antibody [NBP1-86326] - Lane 1: Marker [kDa] 250, 130, 95, 72, 55, 36, 28, 17, 10.Lane 2: Negative control (vector only transfected HEK293T lysate).Lane 3: Over-expression lysate (Co-expressed with a C-terminal myc-DDK tag (~3.1 kDa) in mammalian HEK293T cells, LY405219) Immunohistochemistry-Paraffin: BCKDHB Antibody [NBP1-86326] - Staining of human stomach shows strong cytoplasmic positivity(granular pattern) in glandular cells. 0.1 mL Log in to see 7 to 9 Days
$439.69
Details
Human Rabbit Un-conjugated ELISA, FACS, IF, IHC, WB   200 μL Log in to see 7 to 9 Days
$713.17
Details
Human Rabbit Biotin ELISA, FACS, ICC, IF, IHC, WB   200 μL Log in to see 7 to 9 Days
$969.83
Details
Human Rabbit PE ELISA, FACS, ICC, IF, IHC, WB   200 μL Log in to see 7 to 9 Days
$969.83
Details
Human Rabbit APC ELISA, FACS, ICC, IF, IHC, WB   200 μL Log in to see 7 to 9 Days
$969.83
Details
Human Rabbit HRP ELISA, FACS, ICC, IF, IHC, WB   200 μL Log in to see 7 to 9 Days
$969.83
Details

BCKDHB Antibodies by Reactivity, Application, Clonality and Conjugate

Attributes Applications Host Clonality Conjugate
Rat (Rattus)


Human ,


, , , , ,
Mouse (Murine)


More Antibodies against BCKDHB Interaction Partners

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

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

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

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

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

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

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

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

  6. 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.

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

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

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

  10. 30 Maple syrup urine disease Portuguese patients studied; 17 putative mutations have been identified (6 in BCKDHA (show BCKDHA Antibodies), 5 in BCKDHB and 6 in DBT (show DBT Antibodies)); 7 of are described for the first time.

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

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

BCKDHB Antigen 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) antibody
  • branched chain ketoacid dehydrogenase E1, beta polypeptide (bckdhb) antibody
  • branched chain keto acid dehydrogenase E1, beta polypeptide (bckdhb) antibody
  • branched chain keto acid dehydrogenase E1, beta polypeptide (Bckdhb) antibody
  • branched chain ketoacid dehydrogenase E1, beta polypeptide (Bckdhb) antibody
  • BCKDHB antibody
  • dJ279A18.1 antibody
  • E1B antibody
  • zgc:158777 antibody

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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