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Lipoamide acyltransferase component of branched-chain alpha-keto acid dehydrogenase complex, mitochondrial (DBT)
- Target
- DBT
- Molecular classification
- Enzyme, Transferase, Mitochondrial protein
01
Overview
Lipoamide acyltransferase component of branched-chain alpha-keto acid dehydrogenase complex, mitochondrial (abbreviated DBT), is the E2 transacylase subunit of the mitochondrial branched-chain alpha-keto acid dehydrogenase complex (BCKD). This enzyme complex catalyzes a crucial step in the catabolic breakdown of the branched-chain amino acids leucine, isoleucine, and valine. DBT plays a key role in transferring acyl groups during this metabolic process, allowing the amino acids to be used for energy production. Mutations in the DBT gene disrupt this metabolic step, resulting in the accumulation of toxic metabolites and causing maple syrup urine disease, an inherited metabolic disorder resulting in neurological impairment and potentially life-threatening symptoms if untreated.
- Other names
- Dihydrolipoamide branched chain transacylase E2BCATE2BCKDHE2BCOADC-E2BCKAD-E2BCKDH-E252 kDa mitochondrial autoantigen of primary biliary cirrhosisBranched chain 2-oxo-acid dehydrogenase complex component E2E2 component of branched chain keto acid dehydrogenase complexMSUD2ODB2_HUMAN
02
Mechanism of action
Not applicable for standard drugs; pathology occurs due to loss-of-function mutations, not inhibition
03
Biological functions
Amino acid catabolism (branched-chain amino acids)Energy metabolism
04
Disease associations
Maple syrup urine diseaseInborn errors of metabolismNeurological impairment (as consequence of disease)
05
Safety considerations
Toxic accumulation of branched-chain amino acids and their ketoacids in deficiency statesRisk of neurological damage if untreated in maple syrup urine disease
06
Interacting drugs
None reported in major drug databases; dietary management is main intervention
07
Biomarkers
Elevated concentrations of branched-chain amino acids (leucine, isoleucine, valine)Metabolites in blood or urine indicating maple syrup urine disease (e.g., alloisoleucine)
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