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ZIRC
ZFIN ID: ZDB-GENE-050522-376
Gene Name: branched chain keto acid dehydrogenase E1 subunit alpha
Gene Symbol: bckdha    Nomenclature History

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Previous Names: wu:fd20d04, zgc:110049

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(Including Attributions)
Gene Type: protein_coding_gene
Location: Chr: 18 Mapping Details/Browsers
Description: Predicted to have alpha-ketoacid dehydrogenase activity. Predicted to be involved in branched-chain amino acid catabolic process. Predicted to localize to the 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).
Genome Resources: Alliance (1),  Gene:554124 (1),  Ensembl(GRCz11):ENSDARG00000040555 (1)
GENE EXPRESSION
Directly Submitted Expression Data: 1 figure (1 image) from Thisse et al., 2004 [IMAGE:7158641]
Wild-type Stages, Structures: Zygote:1-cell (0.0h-0.75h) to Hatching:Pec-fin (60.0h-72.0h)
 
Curated Microarray Expression: GEO (1)
PHENOTYPE No data available
DISEASE ASSOCIATED WITH bckdha HUMAN ORTHOLOG
Disease Ontology Term Multi-Species Data OMIM Term OMIM Phenotype ID
maple syrup urine disease Alliance Maple syrup urine disease, type Ia 248600
DISEASE ASSOCIATED WITH bckdha VIA EXPERIMENTAL MODELS No data available
PROTEIN FAMILIES, DOMAINS AND SITES
  • InterPro:IPR001017 (1)
  • InterPro:IPR029061 (1)
  • InterPro:IPR034616 (1)
  • Pfam:PF00676 (1)
  • EC:1.2.4.4 (1)
  • TRANSCRIPTS
    Type Name Length (nt) Analysis
    mRNA bckdha-201 (1)    Ensembl 2368
    Browsers: UCSCNCBIEnsemblZFIN
    INTERACTIONS AND PATHWAYS
    ANTIBODIES No data available
    PLASMIDS No data available
    CONSTRUCTS WITH SEQUENCES FROM bckdha No data available
    MARKER RELATIONSHIPS
    bckdha Contained in: [BAC] CH211-194N6 (1) (order this)
    bckdha Encodes: [EST] fd20d04 (1), IMAGE:7158641 (1) (order this)
    [cDNA] MGC:110049 (1) (order this), MGC:191850 (1) (order this)
    SEQUENCE INFORMATION
    Type Accession # Length (bp/aa) Analysis
    RNA RefSeq:NM_001024419 (1) 1812 bp
    Genomic GenBank:BX649594 (1) 195263 bp
    Select Tool
    Polypeptide UniProtKB:Q4VBU0 (1) 446 aa
    Sequence Clusters UniGene:78707 (1)
    Sequence Information (all 17)
    ORTHOLOGY for bckdha ( Chr: 18 )
    CITATIONS (22)