(R)-methylmalonyl-CoA

1. (R)-Methylmalonyl-CoA CoA-carbonylmutase

Catalysed by MMUTmitochondrial matrix

1. (R)-methylmalonyl-CoA = succinyl-CoA

Catalysed by MMUT

1. (R)-Methylmalonyl-CoA CoA-carbonylmutase
2. 5-aminolevulinate synthase (2-amino-3-oxoadipate)

Catalysed by MMUT, ALAS1, CPNE7mitochondrial matrixOne of 8 routes

1. (R)-Methylmalonyl-CoA CoA-carbonylmutase
2. succinyl-CoA:acetoacetate CoA-transferase

Catalysed by MMUT, OXCT1, OXCT2mitochondrial matrix

1. (R)-Methylmalonyl-CoA CoA-carbonylmutase
2. 5-aminolevulinate synthase (2-amino-3-oxoadipate)
3. Carnitine O-Palmitoyltransferase

Catalysed by MMUT, ALAS1, CPNE7, CPT1A and 2 moremitochondrial matrix, cytosolOne of 244 routes

1. (R)-methylmalonyl-CoA = succinyl-CoA
2. 3-hydroxy-3-methylglutarate + succinyl-CoA = (3S)-3-hydroxy-3-methylglutaryl-CoA + succinate
3. (3S)-3-hydroxy-3-methylglutaryl-CoA = acetoacetate + acetyl-CoA