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DraconiticBiochemistryBiomolecules · Catabolism · Biosynthesis · MetabolismMap
Data
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DBT transfers BCAA to CoA

BCAA-lipoH2-K105-DBT + CoA-SH → BCAA-CoAs + LipoH2-K105-DBT

Reactome
R-HSA-9859163
Compartment
mitochondrial matrix
Rhea
RHEA:18867

Inputs

Outputs

Catalysed by

  • acyltransferase activity, transferring groups other than amino-acyl groups of BCKDH [mitochondrial matrix]EC 2.3.1

Literature

  1. Genomic and biochemical analysis of repeatedly observed variants in DBT in individuals with maple syrup urine disease of Central American ancestry Am J Med Genet A, 2022