. . "~54 hours"@en . . . . . . . "Humans and other mammals"@en . "Nitisinone is a synthetic reversible inhibitor of 4-hydroxyphenylpyruvate dioxygenase. It is used in the treatment of hereditary tyrosinemia type 1. It is sold under the brand name Orfadin. [Wikipedia]"@en . "Nitisinone"@en . "Orfadin"@en . . . . . . . . . . . . . . . . . "Nitisinonum"@en . . "The capsule and liquid formulations are bioequivalent in both the plasma concentration-time curve and maximum plasma concentration (Cmax)."@en . . . . . "Nitisinona"@en . "104206-65-7"@en . "Used as an adjunct to dietary restriction of tyrosine and phenylalanine in the treatment of hereditary tyrosinemia type 1."@en . . . . . . . "approved"@en . . . . . . "Nitisinone is a competitive inhibitor of 4-hydroxyphenyl-pyruvate dioxygenase, an enzyme upstream of fumarylacetoacetate hydrolyase (FAH) 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 catabolic intermediates maleylacetoacetate and fumarylacetoacetate."@en . . . "2-(alpha,alpha,alpha-Trifluoro-2-nitro-P-tuluoyl)-1,3-cyclohexanedione"@en . . . . . . . . . "Side effects include elevated plasma levels of this amino acid, hepatic and liver failure."@en . . . "investigational"@en .