Table 3.

Formulations of essential and non‐essential amino acids from which specific amino acids have been omitted for treatment of metabolic disorders

Disorder	Amino acids omitted
Glutaric aciduria type I	Lysine (and tryptophan reduced)
Homocysteinuria and hypermethioninaemia	Methionine
Hyperlysinaemia	Lysine
Isovaleric acidaemia	Leucine
Maple syrup urine disease	Isoleucine, leucine, and valine
Methylmalonic acidaemia and propionic acidaemia	Methionine, threonine, and valine (and isoleucine reduced)
Phenylketonuria	Phenylalanine
Tyrosinaemia	Phenylalanine and tyrosine