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(S)-methylmalonyl-CoA H+ trans-2-Methylbut-2-enoyl-CoA (S)-3-methyl-2-oxopentanoate thiamine diphosphate NAD+ 2-oxoglutarate CO2 NADH H+ (R)-methylmalonyl-CoA HCO3- lipoamide-E H2O ATP lipoamide-E S-(2-methylbutanoyl)- dihydrolipoamide-E CoA dihydrolipoamide-E H+ thiamine diphosphate FAD Propionyl-CoA H+ FADH2 ADP (2S,3S)-3-hydroxy-2-methylbutanoyl-CoA CoA NAD+ NADH succinyl-CoA acetyl-CoA 2-Methylacetoacetyl-CoA 2-methyl-1-hydroxybutyl-thiamin diphosphate L-glutamate (S)-2-MethylbutanoylCoA L-isoleucine 2.6.1.42 | branched-chain amino acid aminotransferase (isoleucine) 2.3.1.16 | acetyl-CoA:propanoyl-CoA 2-C-acetyltransferase 1.3.99.3 | acyl-CoA dehydrogenase (isoleucine) 1.2.4.4 | 3-methyl-2-oxobutanoate dehydrogenase (E1) (isoleucine) 4.2.1.17 | enoyl-CoA hydratase (isoleucine) 1.8.1.4 | dihydrolipoamide dehydrogenase (E3) (isoleucine) 1.2.4.4 | 3-methyl-2-oxobutanoate dehydrogenase (E1) (isoleucine) 1.1.1.178 | 3-hydroxyacyl-CoA-dehydrogenase 6.4.1.3 | propionyl-CoA carboxylase 2.3.1.168 | 2-methylpropanoyl-CoA:dihydrolipamide-E S-(2-methylpropanoyl) transferase (E2) (isoleucine) 5.4.99.2 | Methylmalonyl-CoA mutase 5.1.99.1 | Methylmalonyl-CoA epimerase
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