| Bioactivity | Nitisinone is an orally active, competitive and reversible 4-hydroxyphenylpyruvate dioxygenase (4-HPPD) inhibitor with an IC50 of 173 nM. Nitisinone promotes tyrosine accumulation in a dose-dependent manner. nitisinone can be used in studies of hereditary tyrosinemia type 1 (HT-1) (a rare genetic disorder) and albinism[1][2][3][4]. | ||||||||||||
| Invitro | Nitisinone (0.01-10 µM; 72 h) promotes tyrosine accumulation in a dose-dependent manner[1]. Cell Viability Assay[1] Cell Line: | ||||||||||||
| In Vivo | Nitisinone (5, 10 mg/kg; p.o.; 5 days aweek for 6 weeks) inhibits HPPD in a dose- and time- dependent manner in rat liver[2].Nitisinone (4 mg/kg; p.o.; single daily; one day interval for 1 month) elevates tyrosine in both OCA-1A and OCA-1B model[3].Nitisinone (4 mg/kg; p.o.; single daily; one day interval for 1 month) increases coat and iris pigmentation and melanin content in the melanosomes of ocular tissues in a mouse model of OCA-1B[3].Note: Oculocutaneous albinism, type 1 (OCA1). There are 2 forms of OCA1, OCA-1A and OCA-1B. Individuals with the former lack functional tyrosinase and therefore lack melanin, while individuals with the latter produce some melanin[3]. Animal Model: | ||||||||||||
| Name | Nitisinone | ||||||||||||
| CAS | 104206-65-7 | ||||||||||||
| Formula | C14H10F3NO5 | ||||||||||||
| Molar Mass | 329.23 | ||||||||||||
| Appearance | Solid | ||||||||||||
| Transport | Room temperature in continental US; may vary elsewhere. | ||||||||||||
| Storage |
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| Reference | [1]. Laschi M, et al. Inhibition of para-Hydroxyphenylpyruvate Dioxygenase by Analogues of the Herbicide Nitisinone As a Strategy to Decrease Homogentisic Acid Levels, the Causative Agent of Alkaptonuria. ChemMedChem. 2016 Apr 5;11(7):674-8. [2]. Ellis MK, et al. Inhibition of 4-hydroxyphenylpyruvate dioxygenase by 2-(2-nitro-4-trifluoromethylbenzoyl)-cyclohexane-1,3-dione and 2-(2-chloro-4-methanesulfonylbenzoyl)-cyclohexane-1,3-dione. Toxicol Appl Pharmacol. 1995 Jul;133(1):12-9. [3]. Onojafe IF, et al. Nitisinone improves eye and skin pigmentation defects in a mouse model of oculocutaneous albinism. J Clin Invest. 2011 Oct;121(10):3914-23. [4]. Aktuglu-Zeybek A, et al. Nitisinone: a review. Orphan Drugs: Research and Reviews, 2017, 7. |
Nitisinone
CAS: 104206-65-7 F: C14H10F3NO5 W: 329.23
Nitisinone is an orally active, competitive and reversible 4-hydroxyphenylpyruvate dioxygenase (4-HPPD) inhibitor with a
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