Nitisinone
Nitisinone Brand names, Nitisinone Analogs
Nitisinone Brand Names Mixture
- No information avaliable
Nitisinone Chemical_Formula
C14H10F3NO5
Nitisinone RX_link
http://www.rxlist.com/cgi/generic/orfadin.htm
Nitisinone fda sheet
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
2.105
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
