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Nitisinone Brand names, Nitisinone Analogs

Nitisinone Brand Names Mixture

  • No information avaliable

Nitisinone Chemical_Formula


Nitisinone RX_link

Nitisinone fda sheet

Nitisinone FDA

Nitisinone msds (material safety sheet)

Nitisinone Synthesis Reference

No information avaliable

Nitisinone Molecular Weight

329.228 g/mol

Nitisinone Melting Point

No information avaliable

Nitisinone H2O Solubility

No information avaliable

Nitisinone State

No information avaliable

Nitisinone LogP


Nitisinone Dosage Forms

Capsules; Liquid

Nitisinone Indication

Used as an adjunct to dietary restriction of tyrosine and phenylalanine in the treatment of hereditary tyrosinemia type 1.

Nitisinone Pharmacology

Hereditary tyrosinemia type 1 occurs due to a deficiency in fumarylacetoacetase (FAH), the final enzyme in the tyrosine catabolic pathway. Nitisinone inhibits catabolism of tyrosine by preventing the catabolic intermediates. In patients with HT-1, these catabolic intermediates are converted to the toxic metabolites succinylacetone and succinylacetoacetate, which are responsible for the observed liver and kidney toxicity. Succinylacetone can also inhibit the porphyrin synthesis pathway leading to the accumulation of 5-aminolevulinate, a neurotoxin responsible for the porphyric crises characteristic of HT-1.

Nitisinone Absorption

The capsule and liquid formulations are bioequivalent in both the plasma concentration-time curve and maximum plasma concentration (Cmax).

Nitisinone side effects and Toxicity

Side effects include elevated plasma levels of this amino acid, hepatic and liver failure.

Nitisinone Patient Information

Nitisinone Organisms Affected

Humans and other mammals