1. Metabolic Enzyme/Protease
  2. HPPD
  3. Nitisinone

Nitisinone  (Synonyms: NTBC; Nitisone; SC0735)

Cat. No.: HY-B0607 Purity: 99.69%
COA Handling Instructions

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.

For research use only. We do not sell to patients.

Nitisinone Chemical Structure

Nitisinone Chemical Structure

CAS No. : 104206-65-7

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Solid + Solvent
10 mM * 1 mL in DMSO
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Solid
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50 mg USD 110 In-stock
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Customer Review

Based on 2 publication(s) in Google Scholar

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Description

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].

In Vitro

Nitisinone (0.01-10 μM; 72 h) promotes tyrosine accumulation in a dose-dependent manner[1].

MedChemExpress (MCE) has not independently confirmed the accuracy of these methods. They are for reference only.

Cell Viability Assay[1]

Cell Line: Human primary fibroblasts (HFb)
Concentration: 0.01-10 µM
Incubation Time: 72 h
Result: Exhibited massive intracellular accumulation of tyrosine in human cell cultures.
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].

MedChemExpress (MCE) has not independently confirmed the accuracy of these methods. They are for reference only.

Animal Model: Male Wistar rats (120-150 g)[2].
Dosage: 5, 10 mg/kg
Administration: Oral gavage; 5 days aweek for 6 weeks.
Result: Inhibited HPPD in a dose- and time- dependent manner in rat liver. (rat liver form animal model, incubate with 0-200 nM Nitisinone for 3 min).
Animal Model: C57BL/6J mice (WT mice), Tyrc-2J/c-2J mice (model of OCA-1A) and Tyrc-h/c-h mice (model of OCA-1B) (all are 3 to 4-month-age)[3].
Dosage: 4 mg/kg
Administration: Oral gavage; single daily; every other day for 1 month.
Result: Elevated plasma tyrosine levels 4- to 6- fold compared with placebo-treated controls, after 1 month in both OCA-1A and OCA-1B model.
Increased pigmentation in the irides and pigmentation in areas of new hair growth upon physical, in OCA-1B model.
Significant increased in the number of pigmented melanosomes in OCA-1B model.
Showed substantial pigmentation in the irides of Tyrc-h/c-h pups born of Nitisinone-treated mothers.
Clinical Trial
Molecular Weight

329.23

Formula

C14H10F3NO5

CAS No.
Appearance

Solid

Color

Light yellow to yellow

SMILES

O=C1C(C(C2=CC=C(C(F)(F)F)C=C2[N+]([O-])=O)=O)C(CCC1)=O

Shipping

Room temperature in continental US; may vary elsewhere.

Storage
Powder -20°C 3 years
4°C 2 years
In solvent -80°C 6 months
-20°C 1 month
Solvent & Solubility
In Vitro: 

DMSO : 41.67 mg/mL (126.57 mM; Need ultrasonic)

Preparing
Stock Solutions
Concentration Solvent Mass 1 mg 5 mg 10 mg
1 mM 3.0374 mL 15.1870 mL 30.3739 mL
5 mM 0.6075 mL 3.0374 mL 6.0748 mL
10 mM 0.3037 mL 1.5187 mL 3.0374 mL
*Please refer to the solubility information to select the appropriate solvent.
In Vivo:
  • 1.

    Add each solvent one by one:  10% DMSO    40% PEG300    5% Tween-80    45% Saline

    Solubility: ≥ 2.5 mg/mL (7.59 mM); Clear solution

  • 2.

    Add each solvent one by one:  10% DMSO    90% Corn Oil

    Solubility: ≥ 2.5 mg/mL (7.59 mM); Clear solution

*All of the co-solvents are available by MedChemExpress (MCE).
Purity & Documentation

Purity: 99.69%

References
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Nitisinone Related Classifications

Help & FAQs
  • Do most proteins show cross-species activity?

    Species cross-reactivity must be investigated individually for each product. Many human cytokines will produce a nice response in mouse cell lines, and many mouse proteins will show activity on human cells. Other proteins may have a lower specific activity when used in the opposite species.

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Nitisinone
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HY-B0607
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