BCKDHB Proteins

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.

Data Quality
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Type
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    Protein
Target
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    Branched Chain Keto Acid Dehydrogenase E1, beta Polypeptide (BCKDHB)
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  • 241
  • 237
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Application
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  • 2
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  • 1
Reactivity
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  • 1
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Host
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Binding Specificity
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Conjugate
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Protein Type
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Format
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Suggested next Filters
6 Products
Recombinant Protein, Insect Cells (Source), Purity >95 % as determined by SDS PAGE, Size Exclusion Chromatography and Western Blot.
ReactivityHuman
Application Crys, ELISA, SDS, WB
Order details Cat. No. ABIN3084865
$6,570.39
Plus shipping costs $45.00 and $22.00 dry ice
Will be delivered in 50 Business Days
Recombinant Protein, Insect Cells (Source), Purity >95 % as determined by SDS PAGE, Size Exclusion Chromatography and Western Blot.
ReactivityMouse
Application Crys, ELISA, SDS, WB
Order details Cat. No. ABIN3123598
$6,570.39
Plus shipping costs $45.00 and $22.00 dry ice
Will be delivered in 50 Business Days
ReactivityHuman
Application AA, AP, ELISA, WB
Order details Cat. No. ABIN2752727
$230.67
2 μg
Plus shipping costs $45.00 and $22.00 dry ice
Delivery in 11 to 12 Business Days
Recombinant Protein, HEK-293 Cells (Source), Purity > 80 % as determined by SDS-PAGE and Coomassie blue staining
ReactivityHuman
Application AbP, Func, PI, STD
Order details Cat. No. ABIN2715188
$888.80
20 μg
Plus shipping costs $45.00 and $22.00 dry ice
Will be delivered in 11 Business Days
ReactivityRat
Application ELISA
Order details Cat. No. ABIN1474395
$2,927.83
1 mg
Plus shipping costs $45.00
Delivery in 60 to 71 Business Days
ReactivityCow
Application ELISA
Order details Cat. No. ABIN1459678
$2,927.83
1 mg
Plus shipping costs $45.00
Delivery in 60 to 71 Business Days
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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) antibody
  • branched chain keto acid dehydrogenase E1 subunit beta (BCKDHB) antibody
  • branched chain keto acid dehydrogenase E1, beta polypeptide (bckdhb) antibody
  • branched chain keto acid dehydrogenase E1, beta polypeptide S homeolog (bckdhb.S) antibody
  • branched chain keto acid dehydrogenase E1 subunit beta (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