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Mitochondria BCKDHB BCKDHA DLD HMGCL BCKDHB BCKDHB BCAT1 BCAT1 BCAT1 BCKDHA BCKDHB DBT IVD ACADM ECHS1 MCCC1 MCCC2 DLD DBT BCKDHA BCKDHB BCKDHA BCKDHB DBT ACADS ACADSB ACAD8 ACADM ECHS1 HIBCH HIBADH ECHS1 ABAT ACADM ACADS ACADSB ECHS1 HSD17B10 ACAA2 ALDH6A1 PCCA PCCB MCEE MUT AOX1 ALDH2 AUH HMGCS2 ACAT1 OXCT1 BCKDHA BCKDHA (S)-beta- Aminoisobutyric acid Acetyl-CoA Propionyl-CoA Acetyl-CoA Succinyl-CoA 3-Methyl-1- hydroxybutyl- ThPP Lipoamide S-(3- Methylbutanoyl)- dihydrolipoamide- E Dihydrolipoamide NAD NADH Hydrogen Ion 3-Hydroxy-3- methylglutaryl- CoA Acetoacetic acid 2-Methyl-1- hydroxypropyl- ThPP S-(2- Methylpropionyl)- dihydrolipoamide- E Lipoamide 2-Methyl-1- hydroxypropyl- ThPP S-(2- Methylbutanoyl)- dihydrolipoamide Thiamine pyrophosphate L-Valine Oxoglutaric acid α- Ketoisovaleric acid L-Glutamic acid L-Leucine Oxoglutaric acid Ketoleucine L-Glutamic acid L-Isoleucine Oxoglutaric acid 3-Methyl-2- oxovaleric acid L-Glutamic acid Thiamine pyrophosphate Carbon dioxide Coenzyme A Isovaleryl-CoA 3- Methylcrotonyl- CoA 3- Hydroxyisovaleryl- CoA Water Adenosine triphosphate Hydrogen carbonate 3- Methylglutaconyl- CoA Adenosine diphosphate Dihydrolipoamide NAD NADH Hydrogen Ion Coenzyme A Isobutyryl-CoA Carbon dioxide Carbon dioxide Coenzyme A 2- Methylbutyryl- CoA Methacrylyl-CoA Water (S)-3- Hydroxyisobutyryl- CoA Water (S)-3- Hydroxyisobutyric acid Coenzyme A NAD (S)- Methylmalonic acid semialdehyde NADH Hydrogen Ion Oxoglutaric acid L-Glutamic acid Tiglyl-CoA Water 2-Methyl-3- hydroxybutyryl- CoA NAD 2- Methylacetoacetyl- CoA NADH Coenzyme A Water NAD Coenzyme A NADH Hydrogen carbonate Adenosine triphosphate Hydrogen carbonate S- Methylmalonyl- CoA Adenosine diphosphate Phosphate R- Methylmalonyl- CoA Water Oxygen Methylmalonic acid Hydrogen peroxide NAD Water NADH Water Acetoacetyl-CoA Water Coenzyme A Coenzyme A Succinyl-CoA Succinic acid electron- transfer flavoprotein Reduced electron- transfer flavoprotein electron- transfer flavoprotein Reduced electron- transfer flavoprotein electron- transfer flavoprotein Reduced electron- transfer flavoprotein Pyrimidine Metabolism Citric Acid Cycle Propanoate Metabolism Citric Acid Cycle Citric Acid Cycle