2. Gene
  3. Bckdhb - branched chain keto acid dehydrogenase E1 subunit beta Gene

Bckdhb - branched chain keto acid dehydrogenase E1 subunit beta Gene

  • Gene
  • Protein
  • Agent
  • Ortholog
Rattus norvegicus

Also known as BCKDE1B

Gene ID: 29711 | Gene type: protein coding

About Bckdhb

Summary

Predicted to contribute to 3-methyl-2-oxobutanoate dehydrogenase (2-methylpropanoyl-transferring) activity. Involved in response to cAMP; response to glucocorticoid; and response to nutrient. Predicted to be located in mitochondrion and nucleolus. Predicted to be part of mitochondrial alpha-ketoglutarate dehydrogenase complex. Human ortholog(s) of this gene implicated in maple syrup urine disease. Orthologous to human BCKDHB (branched chain keto acid dehydrogenase E1 subunit beta). [provided by Alliance of Genome Resources, Apr 2022]

Bckdhb Products(1)

mRNA Protein Name
NM_019267.1 NP_062140.1 2-oxoisovalerate dehydrogenase subunit beta, mitochondrial precursor
Gene Ontology
  • Molecular Function
  • Biological Process
Molecular Function GO Annotation Evidence Reference Source
enables protein-containing complex binding IPI
IPI: Inferred from physical interaction
3619032 RGD
Biological Process GO Annotation Evidence Reference Source
involved in response to cAMP IEP
IEP: Inferred from expression pattern
8034710 RGD
involved in response to glucocorticoid IEP
IEP: Inferred from expression pattern
8034710 RGD
involved in response to nutrient IEP
IEP: Inferred from expression pattern
8109974 RGD
EXP: Inferred from Experiment IDA: Inferred from direct assay IPI: Inferred from physical interaction IMP: Inferred from mutant phenotype IGI: Inferred from genetic interaction IEP: Inferred from expression pattern
Protein Preferred Names Protein Names

2-oxoisovalerate dehydrogenase subunit beta, mitochondrial

2-oxoisovalerate dehydrogenase beta subunit

BCKDH E1-beta

branched chain keto acid dehydrogenase E1 beta

branched chain keto acid dehydrogenase E1, beta polypeptide

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

Related Agents

Pre-clinical Phase
Bioactive Molecules (1)
Cat. No. Product Name Effect
Purity Rare Diseases
HY-RS01410 BCKDHB Human Pre-designed siRNA Set A Pre-designed Set / Unkown

Orthologs Information

Species Symbol Source ID
Homo sapiens Bckdhb NCBI NCBI:594