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Bckdha branched chain keto acid dehydrogenase E1 subunit alpha [ Rattus norvegicus (Norway rat) ]

Gene ID: 25244, updated on 11-Apr-2024

Summary

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Official Symbol
Bckdhaprovided by RGD
Official Full Name
branched chain keto acid dehydrogenase E1 subunit alphaprovided by RGD
Primary source
RGD:2196
See related
Ensembl:ENSRNOG00000020607 AllianceGenome:RGD:2196
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
E1a; BCKDA
Summary
Predicted to enable 3-methyl-2-oxobutanoate dehydrogenase (2-methylpropanoyl-transferring) activity. Involved in response to cAMP; response to glucocorticoid; and response to nutrient. Predicted to be located in mitochondrion. Predicted to be part of mitochondrial alpha-ketoglutarate dehydrogenase complex. Human ortholog(s) of this gene implicated in maple syrup urine disease. Orthologous to human BCKDHA (branched chain keto acid dehydrogenase E1 subunit alpha). [provided by Alliance of Genome Resources, Apr 2022]
Expression
Biased expression in Adrenal (RPKM 1002.9), Kidney (RPKM 697.8) and 9 other tissues See more
Orthologs
human mouse all
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Genomic context

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Location:
1q21
Exon count:
9
Annotation release Status Assembly Chr Location
RS_2024_02 current GRCr8 (GCF_036323735.1) 1 NC_086019.1 (90266731..90295521, complement)
RS_2023_06 previous assembly mRatBN7.2 (GCF_015227675.2) 1 NC_051336.1 (81138946..81167765, complement)
106 previous assembly Rnor_6.0 (GCF_000001895.5) 1 NC_005100.4 (82423291..82452094, complement)

Chromosome 1 - NC_086019.1Genomic Context describing neighboring genes Neighboring gene distal membrane arm assembly component 2 Neighboring gene glutamate-rich 4 Neighboring gene UDP-GlcNAc:betaGal beta-1,3-N-acetylglucosaminyltransferase 8 Neighboring gene exosome component 5 Neighboring gene transmembrane protein 91 Neighboring gene uncharacterized LOC134482996 Neighboring gene B9 domain containing 2

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
  • 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. Downregulation of extramitochondrial BCKDH and its uncoupling from AMP deaminase in type 2 diabetic OLETF rat hearts. Ogawa T, et al. Physiol Rep, 2023 Feb. PMID 36802195, Free PMC Article
  2. Molecular cloning of a cDNA for the E1 alpha subunit of rat liver branched chain alpha-ketoacid dehydrogenase. Zhang B, et al. J Biol Chem, 1987 Nov 5. PMID 2822716
  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. Posttranscriptional alterations in protein masses of hepatic branched-chain keto acid dehydrogenase and its associated kinase in diabetes. Lombardo YB, et al. Proc Assoc Am Physicians, 1998 Jan-Feb. PMID 9460082
  5. Effect of dietary protein on the liver content and subunit composition of the branched-chain alpha-ketoacid dehydrogenase complex. Zhao Y, et al. Arch Biochem Biophys, 1994 Feb 1. PMID 8109974

See all (20) citations in PubMed

GeneRIFs: Gene References Into Functions

What's a GeneRIF?
  1. Downregulation of extramitochondrial BCKDH and its uncoupling from AMP deaminase in type 2 diabetic OLETF rat hearts.
    Title: Downregulation of extramitochondrial BCKDH and its uncoupling from AMP deaminase in type 2 diabetic OLETF rat hearts.
  2. It has been found that NaBut reduces proliferation rate of ERasBcl (E1A, cHa-ras and bcl-2 ) cells significantly weaker than of ERas transformed cells
    Title: [Antiapoptotic gene bcl-2 prevents cellular senescence program reactivation induced by histone deacetylase inhibitor sodium butyrate in E1A and cHa-ras transformed rat fibroblasts].
  3. 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 3-methyl-2-oxobutanoate dehydrogenase (2-methylpropanoyl-transferring) activity ISS
Inferred from Sequence or Structural Similarity
more info
  
enables 3-methyl-2-oxobutanoate dehydrogenase (2-methylpropanoyl-transferring) activity TAS
Traceable Author Statement
more info
 PubMed 
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 branched-chain alpha-keto acid dehydrogenase activity TAS
Traceable Author Statement
more info
 PubMed 
enables metal ion binding IEA
Inferred from Electronic Annotation
more info
  
enables oxidoreductase activity, acting on the aldehyde or oxo group of donors, disulfide as acceptor 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 branched-chain amino acid catabolic process TAS
Traceable Author Statement
more info
 PubMed 
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 IEP
Inferred from Expression Pattern
more info
 PubMed 
Component Evidence Code Pubs
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 alpha, mitochondrial
Names
BCKDE1A
BCKDH E1-alpha
branched chain alpha-ketoacid dehydrogenase subunit E1 alpha
branched chain keto acid dehydrogenase subunit E1, alpha polypeptide
branched chain ketoacid dehydrogenase E1, alpha polypeptide
branched-chain alpha-keto acid dehydrogenase E1 component alpha chain
NP_036914.1
XP_017444303.1
XP_038957453.1
XP_063137592.1

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_012782.1 → NP_036914.1  2-oxoisovalerate dehydrogenase subunit alpha, mitochondrial

    See identical proteins and their annotated locations for NP_036914.1

    Status: VALIDATED

    Source sequence(s)
    BC089915, CB579195, J02827
    UniProtKB/Swiss-Prot
    P11960
    UniProtKB/TrEMBL
    B1WBN3
    Related
    ENSRNOP00000027995.6, ENSRNOT00000027995.7
    Conserved Domains (1) summary
    pfam00676
    Location:107 → 407
    E1_dh; Dehydrogenase E1 component

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_086019.1 Reference GRCr8

    Range
    90266731..90295521 complement
    Download
    GenBank, FASTA, Sequence Viewer (Graphics)

mRNA and Protein(s)

  1. XM_039101525.2 → XP_038957453.1  2-oxoisovalerate dehydrogenase subunit alpha, mitochondrial isoform X2

    UniProtKB/Swiss-Prot
    P11960
    Conserved Domains (1) summary
    cd02000
    Location:106 → 329
    TPP_E1_PDC_ADC_BCADC; Thiamine pyrophosphate (TPP) family, E1 of PDC_ADC_BCADC subfamily, TPP-binding module; composed of proteins similar to the E1 components of the human pyruvate dehydrogenase complex (PDC), the acetoin dehydrogenase complex (ADC) and the branched chain ...

  2. XM_017588814.3 → XP_017444303.1  2-oxoisovalerate dehydrogenase subunit alpha, mitochondrial isoform X1

    UniProtKB/TrEMBL
    A6J973

  3. XM_063281522.1 → XP_063137592.1  2-oxoisovalerate dehydrogenase subunit alpha, mitochondrial isoform X3

Nucleotide Protein Strain
Heading Accession and Version
Protein Accession Links
GenPept Link UniProtKB Link
P11960.1 GenPept UniProtKB/Swiss-Prot:P11960

Gene LinkOut

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