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Price of Nitisinone
Orfadin 2 mg capsule
* price without discount in nearest pharmacy. Price may vary.
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Nitisinone volume of distribution
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
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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