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RX BIN: 015558
Group ID: DDN6600
Card Holder ID: DDN6600

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2018 Price of Nitisinone

Orfadin 2 mg capsule



* price without discount in nearest pharmacy. Price may vary.

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Nitisinone volume of distribution

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What is Nitisinone

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)

Nitisinone mechanism of action

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.

Dosage forms of Nitisinone

Capsuleoral10 mg
Capsuleoral2 mg
Capsuleoral5 mg
Prescription Generics


International Brands



2-(alpha,alpha,alpha-Trifluoro-2-nitro-P-tuluoyl)-1,3-cyclohexanedione Nitisinona


Mendelikabs Inc

CAS number






Affected organisms

Humans and other mammals

Indication of Nitisinone

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

Toxicity of Nitisinone

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

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