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BCKDHA branched chain keto acid dehydrogenase E1 subunit alpha [ Bos taurus (domestic cattle) ]

Gene ID: 282149, updated on 17-Aug-2024

Summary

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Official Symbol
BCKDHAprovided by VGNC
Official Full Name
branched chain keto acid dehydrogenase E1 subunit alphaprovided by VGNC
Primary source
VGNC:VGNC:108964
See related
BGD:BT12097; Ensembl:ENSBTAG00000016037
Gene type
protein coding
RefSeq status
PROVISIONAL
Organism
Bos taurus
Lineage
Eukaryota; Metazoa; Chordata; Craniata; Vertebrata; Euteleostomi; Mammalia; Eutheria; Laurasiatheria; Artiodactyla; Ruminantia; Pecora; Bovidae; Bovinae; Bos
Annotation information
Annotation category: partial on reference assembly
Orthologs
human mouse all
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Genomic context

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Location:
chromosome: 18
Exon count:
9
Annotation release Status Assembly Chr Location
RS_2023_09 current ARS-UCD2.0 (GCF_002263795.3) 18 NC_037345.1 (50541524..50560679)

Chromosome 18 - NC_037345.1Genomic Context describing neighboring genes Neighboring gene transmembrane protein 91 Neighboring gene exosome component 5 Neighboring gene uncharacterized LOC107131418 Neighboring gene UDP-GlcNAc:betaGal beta-1,3-N-acetylglucosaminyltransferase 8 Neighboring gene distal membrane arm assembly component 2

Genomic regions, transcripts, and products

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Genomic Sequence:
NC_037345.1 Chromosome 18 Reference ARS-UCD2.0 Primary Assembly

Go to nucleotide: Graphics FASTA GenBank

Bibliography

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Related articles in PubMed

  1. Isolation and sequencing of a cDNA encoding the decarboxylase (E1)alpha precursor of bovine branched-chain alpha-keto acid dehydrogenase complex. Expression of E1 alpha mRNA and subunit in maple-syrup-urine-disease and 3T3-L1 cells. Hu CW, et al. J Biol Chem, 1988 Jun 25. PMID 3379058
  2. A whole-genome assembly of the domestic cow, Bos taurus. Zimin AV, et al. Genome Biol, 2009. PMID 19393038, Free PMC Article

GeneRIFs: Gene References Into Functions

Pathways from PubChem

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General gene information

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Markers

Homology

Gene Ontology Provided by RefSeq

Function Evidence Code Pubs
enables oxidoreductase activity, acting on the aldehyde or oxo group of donors, disulfide as acceptor IEA
Inferred from Electronic Annotation
more info
 PubMed 
Process Evidence Code Pubs
involved_in branched-chain amino acid catabolic process IEA
Inferred from Electronic Annotation
more info
 PubMed 

General protein information

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Preferred Names
2-oxoisovalerate dehydrogenase subunit alpha, mitochondrial
Names
BCKDE1A
BCKDH E1-alpha
branched chain keto acid dehydrogenase E1, alpha polypeptide (maple syrup urine disease)
branched-chain alpha-keto acid dehydrogenase E1 component alpha chain
NP_776931.1

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_174506.1 → NP_776931.1  2-oxoisovalerate dehydrogenase subunit alpha, mitochondrial precursor

    See identical proteins and their annotated locations for NP_776931.1

    Status: PROVISIONAL

    Source sequence(s)
    J03759
    UniProtKB/Swiss-Prot
    P11178
    UniProtKB/TrEMBL
    Q2TBT9
    Related
    ENSBTAP00000021342.6, ENSBTAT00000021342.7
    Conserved Domains (1) summary
    pfam00676
    Location:116 → 416
    E1_dh; Dehydrogenase E1 component

RefSeqs of Annotated Genomes: GCF_002263795.3-RS_2023_09

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

Reference ARS-UCD2.0 Primary Assembly

Genomic

  1. NC_037345.1 Reference ARS-UCD2.0 Primary Assembly

    Range
    50541524..50560679
    Download
    GenBank, FASTA, Sequence Viewer (Graphics)
Nucleotide Protein Strain
Heading Accession and Version
Protein Accession Links
GenPept Link UniProtKB Link
P11178.1 GenPept UniProtKB/Swiss-Prot:P11178

Gene LinkOut

The following LinkOut resources are supplied by external providers. These providers are responsible for maintaining the links.

Molecular Biology Databases
Research Materials