Figure 1

Tyrosine metabolic pathway highlighting the enzyme defects responsible and the site of action of nitisinone. i—tyrosine aminotransferase, ii—4-hydroxyphenylpyruvate dioxygenase, iii—homogentisate 1,2-dioxygenase, iv—maleylacetoacetate isomerase, v—fumarylacetoacetate hydrolase.