ATC Group: A16AX04 Nitisinone

Anatomical Therapeutic Chemical Classification System

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Nitisinone

Hierarchical Position

Active Ingredients

Chemical substance
Description

Nitisinone is a competitive inhibitor of 4-hydroxyphenylpyruvate dioxygenase, an enzyme which precedes fumarylacetoacetate hydrolase in the tyrosine catabolic pathway. By inhibiting the normal catabolism of tyrosine in patients with hereditary tyrosinemia type 1 (HT-1), nitisinone prevents the accumulation of the toxic intermediates maleylacetoacetate and fumarylacetoacetate.