Organic acidurias

Isobutyryl-CoA dehydrogenase deficiency
2-Methylbutyryl-CoA dehydrogenase deficiency (Short/branched-chain acyl-CoA dehydrogenase deficiency; 2-methylbutyrylglycinuria)
3-Methylcrotonyl-CoA carboxylase 1 deficiency (3-methylcrotonylglycinuria type 1)
3-Methylcrotonyl-CoA carboxylase 2 deficiency (3-methylcrotonylglycinuria type 2)
3-methylglutaconyl-CoA hydratase deficiency (3-methylglutaconic aciduria type 1)
Mitochondrial short-chain enoyl-CoA hydratase 1 deficiency (Crotonase deficiency)
3-hydroxyisobutyryl-CoA hydrolase deficiency (β-hydroxyisobutyryl-CoA deacylase deficiency)
3-hydroxyisobutyrate dehydrogenase deficiency
Methylmalonate semialdehyde dehydrogenase deficiency
Propionic acidemia due to propionyl-CoA carboxylase subunit alpha deficiency
Propionic acidemia due to propionyl-CoA carboxylase subunit beta deficiency
Methylmalonic aciduria due to methylmalonyl-CoA epimerase deficiency
Methylmalonic aciduria due to methylmalonyl-CoA mutase deficiency
Glutaryl-CoA dehydrogenase deficiency (Glutaric acidemia type 1)
Succinate-hydroxymethylglutarate-CoA transferase deficiency (Glutaric acidemia type 3)
Malonyl-CoA decarboxylase deficiency(Malonic aciduria)

Aminoacid and organic acids related disorders