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 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)
 
High Throughput Expression: GEO (1) , Expression Atlas (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 No links to external sites available
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)
bckdha Encodes: [EST] fd20d04 (1), IMAGE:7158641 (1)
[cDNA] MGC:110049 (1), MGC:191850 (1)
SEQUENCE INFORMATION
Type Accession # Length (nt/aa) Analysis
RNA RefSeq:NM_001024419 (1) 1812 nt
Genomic GenBank:BX649594 (1) 195263 nt
Select Tool
Polypeptide UniProtKB:Q4VBU0 (1) 446 aa
Sequence Information (all 16)
ORTHOLOGY for bckdha ( Chr: 18 )
CITATIONS (23)