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Bckdhb branched chain keto acid dehydrogenase E1 subunit beta [ Rattus norvegicus (Norway rat) ]

Gene ID: 29711, updated on 2-Nov-2024

Summary

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Official Symbol
Bckdhbprovided by RGD
Official Full Name
branched chain keto acid dehydrogenase E1 subunit betaprovided by RGD
Primary source
RGD:2197
See related
EnsemblRapid:ENSRNOG00000009928 AllianceGenome:RGD:2197
Gene type
protein coding
RefSeq status
VALIDATED
Organism
Rattus norvegicus
Lineage
Eukaryota; Metazoa; Chordata; Craniata; Vertebrata; Euteleostomi; Mammalia; Eutheria; Euarchontoglires; Glires; Rodentia; Myomorpha; Muroidea; Muridae; Murinae; Rattus
Also known as
BCKDE1B
Summary
Predicted to enable metal ion binding activity. Predicted to contribute to 3-methyl-2-oxobutanoate dehydrogenase (2-methylpropanoyl-transferring) activity and branched-chain alpha-keto acid dehydrogenase activity. Involved in response to cAMP; response to glucocorticoid; and response to nutrient. Predicted to be located in mitochondrion; nucleolus; and nucleoplasm. Predicted to be part of branched-chain alpha-ketoacid dehydrogenase complex and oxoglutarate dehydrogenase complex. Human ortholog(s) of this gene implicated in maple syrup urine disease. Orthologous to human BCKDHB (branched chain keto acid dehydrogenase E1 subunit beta). [provided by Alliance of Genome Resources, Nov 2024]
Expression
Biased expression in Kidney (RPKM 470.9), Heart (RPKM 468.9) and 9 other tissues See more
Orthologs
human mouse all
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Genomic context

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See Bckdhb in Genome Data Viewer
Location:
8q31
Exon count:
11
Annotation release Status Assembly Chr Location
RS_2024_02 current GRCr8 (GCF_036323735.1) 8 NC_086026.1 (93725277..93907799)
RS_2023_06 previous assembly mRatBN7.2 (GCF_015227675.2) 8 NC_051343.1 (84845264..85027812)
106 previous assembly Rnor_6.0 (GCF_000001895.5) 8 NC_005107.4 (91464229..91656134)

Chromosome 8 - NC_086026.1Genomic Context describing neighboring genes Neighboring gene uncharacterized LOC108351756 Neighboring gene uncharacterized LOC102557516 Neighboring gene U2 spliceosomal RNA Neighboring gene mitochondrial ribosomal protein L41 like 1

Genomic regions, transcripts, and products

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Go to nucleotide: Graphics FASTA GenBank

Expression

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  • Project title: A rat RNA-Seq transcriptomic BodyMap across 11 organs and 4 developmental stages A rat RNA-Seq transcriptomic BodyMap across 11 organs and 4 developmental stages
  • Description: 320 RNA samples isolated from 11 organs (adrenal gland, brain, heart, kidney, liver, lung, muscle, spleen, thymus, and testes or uterus) from both sexes of Fischer 344 rats across four developmental stages (2-, 6-, 21-, and 104-weeks-old)
  • BioProject: PRJNA238328
  • Publication: PMID 24510058
  • Analysis date: Mon Jun 6 17:44:12 2016

Bibliography

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Related articles in PubMed

  1. Molecular cloning of the E1 beta subunit of the rat branched chain alpha-ketoacid dehydrogenase. Zhao Y, et al. Biochim Biophys Acta, 1992 Sep 24. PMID 1390893
  2. Molecular cloning of the E1 beta subunit of the rat branched chain alpha-ketoacid dehydrogenase. Zhao Y, et al. Biochim Biophys Acta, 1995 Jan 25. PMID 7841205
  3. Simvastatin increases liver branched-chain α-ketoacid dehydrogenase activity in rats fed with low protein diet. Knapik-Czajka M. Toxicology, 2014 Nov 5. PMID 25193403
  4. Maple syrup urine disease. Complete defect of the E1 beta subunit of the branched chain alpha-ketoacid dehydrogenase complex due to a deletion of an 11-bp repeat sequence which encodes a mitochondrial targeting leader peptide in a family with the disease. Nobukuni Y, et al. J Clin Invest, 1991 May. PMID 2022752, Free PMC Article
  5. Purification of branched chain alpha-ketoacid dehydrogenase complex from rat liver. Shimomura Y, et al. Anal Biochem, 1987 May 15. PMID 3619032

See all (17) citations in PubMed

GeneRIFs: Gene References Into Functions

What's a GeneRIF?
  1. Simvastatin stimulates liver BCKDH activity and BCAAs degradation in rats fed with the low protein diet.
    Title: Simvastatin increases liver branched-chain α-ketoacid dehydrogenase activity in rats fed with low protein diet.
Submit: New GeneRIF Correction

Pathways from PubChem

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Interactions

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Products Interactant Other Gene Complex Source Pubs Description

General gene information

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Markers

Homology

Gene Ontology Provided by RGD

Function Evidence Code Pubs
enables 3-methyl-2-oxobutanoate dehydrogenase (2-methylpropanoyl-transferring) activity IEA
Inferred from Electronic Annotation
more info
  
contributes_to 3-methyl-2-oxobutanoate dehydrogenase (2-methylpropanoyl-transferring) activity ISO
Inferred from Sequence Orthology
more info
  
enables 3-methyl-2-oxobutanoate dehydrogenase (2-methylpropanoyl-transferring) activity ISO
Inferred from Sequence Orthology
more info
  
contributes_to 3-methyl-2-oxobutanoate dehydrogenase (2-methylpropanoyl-transferring) activity ISS
Inferred from Sequence or Structural Similarity
more info
  
enables branched-chain alpha-keto acid dehydrogenase activity IEA
Inferred from Electronic Annotation
more info
  
contributes_to branched-chain alpha-keto acid dehydrogenase activity ISO
Inferred from Sequence Orthology
more info
  
enables metal ion binding IEA
Inferred from Electronic Annotation
more info
  
enables protein-containing complex binding IPI
Inferred from Physical Interaction
more info
 PubMed 
Process Evidence Code Pubs
involved_in branched-chain amino acid catabolic process IBA
Inferred from Biological aspect of Ancestor
more info
  
involved_in branched-chain amino acid catabolic process IEA
Inferred from Electronic Annotation
more info
  
involved_in branched-chain amino acid catabolic process ISO
Inferred from Sequence Orthology
more info
  
involved_in branched-chain amino acid catabolic process ISS
Inferred from Sequence or Structural Similarity
more info
  
involved_in lipid metabolic process IEA
Inferred from Electronic Annotation
more info
  
involved_in response to cAMP IEP
Inferred from Expression Pattern
more info
 PubMed 
involved_in response to glucocorticoid IEP
Inferred from Expression Pattern
more info
 PubMed 
involved_in response to nutrient IBA
Inferred from Biological aspect of Ancestor
more info
  
involved_in response to nutrient IEP
Inferred from Expression Pattern
more info
 PubMed 
Component Evidence Code Pubs
part_of branched-chain alpha-ketoacid dehydrogenase complex IEA
Inferred from Electronic Annotation
more info
  
part_of branched-chain alpha-ketoacid dehydrogenase complex ISO
Inferred from Sequence Orthology
more info
  
part_of branched-chain alpha-ketoacid dehydrogenase complex ISS
Inferred from Sequence or Structural Similarity
more info
  
located_in mitochondrial matrix IEA
Inferred from Electronic Annotation
more info
  
located_in mitochondrion IEA
Inferred from Electronic Annotation
more info
  
located_in mitochondrion ISO
Inferred from Sequence Orthology
more info
  
located_in mitochondrion ISS
Inferred from Sequence or Structural Similarity
more info
  
located_in nucleolus IEA
Inferred from Electronic Annotation
more info
  
located_in nucleolus ISO
Inferred from Sequence Orthology
more info
  
located_in nucleoplasm IEA
Inferred from Electronic Annotation
more info
  
located_in nucleoplasm ISO
Inferred from Sequence Orthology
more info
  
part_of oxoglutarate dehydrogenase complex IBA
Inferred from Biological aspect of Ancestor
more info
  

General protein information

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Preferred Names
2-oxoisovalerate dehydrogenase subunit beta, mitochondrial
Names
2-oxoisovalerate dehydrogenase beta subunit
BCKDH E1-beta
branched chain keto acid dehydrogenase E1 beta
branched chain keto acid dehydrogenase E1, beta polypeptide
branched-chain alpha-keto acid dehydrogenase E1 component beta chain
NP_062140.1

NCBI Reference Sequences (RefSeq)

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RefSeqs maintained independently of Annotated Genomes

These reference sequences exist independently of genome builds. Explain

These reference sequences are curated independently of the genome annotation cycle, so their versions may not match the RefSeq versions in the current genome build. Identify version mismatches by comparing the version of the RefSeq in this section to the one reported in Genomic regions, transcripts, and products above.

mRNA and Protein(s)

  1. NM_019267.1 → NP_062140.1  2-oxoisovalerate dehydrogenase subunit beta, mitochondrial precursor

    See identical proteins and their annotated locations for NP_062140.1

    Status: VALIDATED

    Source sequence(s)
    BM385109, CB547976, CK473898, DV215445, M94040
    UniProtKB/Swiss-Prot
    P35738
    UniProtKB/TrEMBL
    A0A0A0MXW1, A6I1S6
    Related
    ENSRNOP00000013249.7, ENSRNOT00000013249.8
    Conserved Domains (3) summary
    cd07036
    Location:73 → 239
    TPP_PYR_E1-PDHc-beta_like; Pyrimidine (PYR) binding domain of the beta subunits of the E1 components of human pyruvate dehydrogenase complex (E1- PDHc) and related proteins
    PTZ00182
    Location:64 → 388
    PTZ00182; 3-methyl-2-oxobutanate dehydrogenase; Provisional
    pfam02780
    Location:260 → 379
    Transketolase_C; Transketolase, C-terminal domain

RefSeqs of Annotated Genomes: GCF_036323735.1-RS_2024_02

The following sections contain reference sequences that belong to a specific genome build. Explain

Reference GRCr8

Genomic

  1. NC_086026.1 Reference GRCr8

    Range
    93725277..93907799
    Download
    GenBank, FASTA, Sequence Viewer (Graphics)
Nucleotide Protein Strain
Heading Accession and Version
Protein Accession Links
GenPept Link UniProtKB Link
P35738.3 GenPept UniProtKB/Swiss-Prot:P35738

Gene LinkOut

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