2. Gene
  3. Bckdha - branched chain ketoacid dehydrogenase E1, alpha polypeptide Gene

Bckdha - branched chain ketoacid dehydrogenase E1, alpha polypeptide Gene

  • Gene
  • Protein
  • Agent
  • Ortholog
Mus musculus

Also known as BCKAD; E1[a]; BCKDE1A

Gene ID: 12039 | Gene type: protein coding

About Bckdha

Summary

Predicted to enable 3-methyl-2-oxobutanoate dehydrogenase (2-methylpropanoyl-transferring) activity. Predicted to be involved in branched-chain amino acid catabolic process. Located in 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). [provided by Alliance of Genome Resources, Apr 2022]

Bckdha Products(10)

mRNA Protein Name
NM_001403510.1 NP_001390439.1 2-oxoisovalerate dehydrogenase subunit alpha, mitochondrial isoform 1
NM_001403511.1 NP_001390440.1 2-oxoisovalerate dehydrogenase subunit alpha, mitochondrial isoform 3
NM_001403512.1 NP_001390441.1 2-oxoisovalerate dehydrogenase subunit alpha, mitochondrial isoform 3
NM_001403513.1 NP_001390442.1 2-oxoisovalerate dehydrogenase subunit alpha, mitochondrial isoform 3
NM_007533.6 NP_031559.3 2-oxoisovalerate dehydrogenase subunit alpha, mitochondrial isoform 2
NM_001403510.1 NP_001390439.1 2-oxoisovalerate dehydrogenase subunit alpha, mitochondrial isoform 1
NM_001403511.1 NP_001390440.1 2-oxoisovalerate dehydrogenase subunit alpha, mitochondrial isoform 3
NM_001403512.1 NP_001390441.1 2-oxoisovalerate dehydrogenase subunit alpha, mitochondrial isoform 3
NM_001403513.1 NP_001390442.1 2-oxoisovalerate dehydrogenase subunit alpha, mitochondrial isoform 3
NM_007533.6 NP_031559.3 2-oxoisovalerate dehydrogenase subunit alpha, mitochondrial isoform 2
Protein Preferred Names Protein Names

2-oxoisovalerate dehydrogenase subunit alpha, mitochondrial

BCKAD E1[a]

BCKDH E1-alpha

branched-chain alpha-keto acid dehydrogenase E1 component alpha chain

Related Agents

Pre-clinical Phase
Bioactive Molecules (1)
Cat. No. Product Name Effect
Purity Rare Diseases
HY-RS01409 BCKDHA Human Pre-designed siRNA Set A Pre-designed Set / Unkown

Orthologs Information

Species Symbol Source ID
Homo sapiens Bckdha NCBI NCBI:593