| Maple syrup urine disease (MSUD) |
| Isovaleric acidemia (IVA) |
| Methylmalonic aciduria (MMA) |
| Propionic acidemia |
| 3-Hydroxy-3-methylglutaryl-CoA lyase deficiency |
| 3-Methylcrotonylglycinuria |
| Mitochondrial respiratory chain deficiencies (MRCD) |
| Disorders of fatty-acid oxidation |
| 3-Methylglutaconic aciduria (MGCA) |
| 2-Methyl-3-hydroxybutyryl-CoA dehydrogenase (MHBD) deficiency |
| Alpha-methylacetoacetic aciduria |
| Succinyl CoA:3-oxoacid CoA transferase (SCOT) deficiency |
| HMG-CoA synthase (HMGCS) deficiency |
| Isobutyryl-CoA dehydrogenase (IBD) deficiency |
