(2E)-butenoyl-CoA

1. (S)-3-hydroxybutanoyl-CoA hydro-lyase

Catalysed by ECHS1mitochondrial matrixOne of 2 routes

1. glutaryl-CoA + oxidized [electron-transfer flavoprotein] + 2 H(+) = (2E)-butenoyl-CoA + reduced [electron-transfer flavoprotein] + CO2

Catalysed by GCDH

1. glutaryl-CoA + oxidized [electron-transfer flavoprotein] + 2 H(+) = (2E)-butenoyl-CoA + reduced [electron-transfer flavoprotein] + CO2
2. Production of Glutaryl Carnitine

Catalysed by GCDH, CPT1A, CPT1C, CPT1Bcytosol

1. (S)-3-hydroxybutanoyl-CoA hydro-lyase
2. (S)-3-Hydroxybutanoyl-CoA:NAD+ oxidoreductase

Catalysed by ECHS1, ACAA1, HSD17B4, EHHADHmitochondrial matrix, peroxisome

1. glutaryl-CoA + oxidized [electron-transfer flavoprotein] + 2 H(+) = (2E)-butenoyl-CoA + reduced [electron-transfer flavoprotein] + CO2
2. Production of Glutaryl Carnitine
3. Carnitine O-Palmitoyltransferase

Catalysed by GCDH, CPT1A, CPT1C, CPT1BcytosolOne of 244 routes

1. (S)-3-hydroxybutanoyl-CoA hydro-lyase
2. (3S)-3-hydroxybutanoyl-CoA + NAD(+) = acetoacetyl-CoA + NADH + H(+)
3. acetoacetyl-CoA + H2O = acetoacetate + CoA + H(+)