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

Gene ID: 25244, updated on 4-Jan-2025

Summary

Official Symbol
Bckdhaprovided by RGD
Official Full Name
branched chain keto acid dehydrogenase E1 subunit alphaprovided by RGD
Primary source
RGD:2196
See related
EnsemblRapid: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 metal ion binding activity and oxidoreductase activity, acting on the aldehyde or oxo group of donors, disulfide as acceptor. 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. 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 BCKDHA (branched chain keto acid dehydrogenase E1 subunit alpha). [provided by Alliance of Genome Resources, Jan 2025]
Expression
Biased expression in Adrenal (RPKM 1002.9), Kidney (RPKM 697.8) and 9 other tissues See more
Orthologs
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Genomic context

See Bckdha in Genome Data Viewer
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

Expression

  • 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

GeneRIFs: Gene References Into Functions

What's a GeneRIF?

Pathways from PubChem

Interactions

General gene information

Gene Ontology Provided by RGD

Function Evidence Code Pubs
enables 3-methyl-2-oxobutanoate dehydrogenase (2-methylpropanoyl-transferring) activity  
contributes_to 3-methyl-2-oxobutanoate dehydrogenase (2-methylpropanoyl-transferring) activity  
enables 3-methyl-2-oxobutanoate dehydrogenase (2-methylpropanoyl-transferring) activity  
contributes_to 3-methyl-2-oxobutanoate dehydrogenase (2-methylpropanoyl-transferring) activity  
enables 3-methyl-2-oxobutanoate dehydrogenase (2-methylpropanoyl-transferring) activity  
enables 3-methyl-2-oxobutanoate dehydrogenase (2-methylpropanoyl-transferring) activity PubMed 
enables branched-chain alpha-keto acid dehydrogenase activity  
contributes_to branched-chain alpha-keto acid dehydrogenase activity  
enables branched-chain alpha-keto acid dehydrogenase activity PubMed 
enables metal ion binding  
enables oxidoreductase activity, acting on the aldehyde or oxo group of donors, disulfide as acceptor  
enables protein-containing complex binding PubMed 
Component Evidence Code Pubs
part_of branched-chain alpha-ketoacid dehydrogenase complex  
part_of branched-chain alpha-ketoacid dehydrogenase complex  
part_of branched-chain alpha-ketoacid dehydrogenase complex  
located_in mitochondrial matrix  
located_in mitochondrion PubMed 
part_of oxoglutarate dehydrogenase complex  

General protein information

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_012782.1NP_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
    ENSRNOP00000109641.1, ENSRNOT00000151777.1
    Conserved Domains (1) summary
    pfam00676
    Location:107407
    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_039101525.2XP_038957453.1  2-oxoisovalerate dehydrogenase subunit alpha, mitochondrial isoform X2

    UniProtKB/Swiss-Prot
    P11960
    Conserved Domains (1) summary
    cd02000
    Location:106329
    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_017588814.3XP_017444303.1  2-oxoisovalerate dehydrogenase subunit alpha, mitochondrial isoform X1

    UniProtKB/TrEMBL
    A6J973
  3. XM_063281522.1XP_063137592.1  2-oxoisovalerate dehydrogenase subunit alpha, mitochondrial isoform X3

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