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Gene: Bckdha (branched chain ketoacid dehydrogenase E1, alpha polypeptide) Mus musculus
Symbol: Bckdha
Name: branched chain ketoacid dehydrogenase E1, alpha polypeptide
Description: Predicted to have alpha-ketoacid dehydrogenase activity. Predicted to be involved in branched-chain amino acid catabolic process. Localizes to mitochondrion. Is expressed in several structures, including alimentary system; cardiovascular system; central nervous system; genitourinary system; and sensory organ. Human ortholog(s) of this gene implicated in maple syrup urine disease. Orthologous to human BCKDHA (branched chain keto acid dehydrogenase E1 subunit alpha); PARTICIPATES IN 3-hydroxy-3-methylglutaryl-CoA lyase deficiency pathway; 3-hydroxyisobutyric aciduria pathway; 3-methylcrotonyl CoA carboxylase 1 deficiency pathway; INTERACTS WITH 1,1-dichloroethene; 17alpha-ethynylestradiol; 2,3,7,8-tetrabromodibenzodioxine.
Type: protein-coding
RefSeq Status: VALIDATED
Also known as: 2-oxoisovalerate dehydrogenase subunit alpha, mitochondrial; BCKAD E1[a]; BCKDE1A; BCKDH E1-alpha; branched-chain alpha-keto acid dehydrogenase E1 component alpha chain
Related Pseudogenes: Gm19159  
Latest Assembly: GRCm38 - Mouse Genome Assembly GRCm38
Mouse AssemblyChrPosition (strand)SourceGenome Browsers
GRCm38.p6 Ensembl725,629,946 - 25,658,981 (-)EnsemblGRCm38mm10GRCm38
GRCm38725,629,852 - 25,658,782 (-)NCBIGRCm38GRCm38mm10GRCm38
MGSCv37726,414,871 - 26,443,780 (-)NCBIGRCm37mm9NCBIm37
MGSCv36725,338,706 - 25,367,498 (-)NCBImm8
Celera720,238,873 - 20,267,674 (-)NCBICelera
Cytogenetic Map7A3NCBI
JBrowse: View Region in Genome Browser (JBrowse)

Disease Annotations
Gene-Chemical Interaction Annotations
Gene Ontology Annotations
Molecular Pathway Annotations
Phenotype Annotations
References - curated
References - uncurated


Comparative Map Data
Position Markers
QTLs in Region (GRCm38)
miRNA Target Status



Nucleotide Sequences
Protein Sequences

Additional Information

External Database Links
More on Bckdha
Alliance Gene
Ensembl Gene
JBrowse: mm9 mm10
MGI Report
NCBI Genome Data Viewer

RGD Object Information
RGD ID: 10228
Created: 2000-05-25
Species: Mus musculus
Last Modified: 2020-06-30
Status: ACTIVE


RGD is funded by grant HL64541 from the National Heart, Lung, and Blood Institute on behalf of the NIH.