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CAS RN: 104206-65-7 | Product Number: N1054
Nitisinone
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Purity: >98.0%(T)(HPLC)
Synonyms:
- 2-[2-Nitro-4-(trifluoromethyl)benzoyl]-1,3-cyclohexanedione
- 2-[2-Nitro-4-(trifluoromethyl)benzoyl]cyclohexane-1,3-dione
Product Documents:
| Size | Unit Price | Belgium | Japan* |
Shipping Information
|
|---|---|---|---|---|
| 100MG |
€54.00
|
1 | ≥60 |
* Stock available in Belgium: Shipment on the same day
* Stock available in Japan: Please check the Shipping Simulation for estimated shipments. (excludes regulated items and dry ice shipments)
| Product Number | N1054 |
Purity / Analysis Method 
|
>98.0%(T)(HPLC) |
| Molecular Formula / Molecular Weight | C__1__4H__1__0F__3NO__5 = 329.23 |
| Physical State (20 deg.C) | Solid |
| Storage Temperature
|
Refrigerated (0-10°C) |
| Condition to Avoid | Heat Sensitive |
| Packaging and Container
|
100MG-Glass Bottle with Plastic Insert (View image) |
| CAS RN | 104206-65-7 |
| Reaxys Registry Number | 8639943 |
| PubChem Substance ID | 468592462 |
| MDL Number | MFCD01752192 |
Specifications
| Appearance | White to Light yellow powder to crystal |
| Purity(HPLC) | min. 98.0 area% |
| Purity(Neutralization titration) | min. 98.0 % |
| NMR | confirm to structure |
Properties (reference)
| Melting Point | 138 °C |
| Solubility in water | Insoluble |
| Solubility (slightly sol. in) | Methanol, Ether |
GHS
| Pictogram |
|
| Signal Word | Warning |
| Hazard Statements | H302 : Harmful if swallowed. H361 : Suspected of damaging fertility or the unborn child. H362 : May cause harm to breast-fed children. |
| Precautionary Statements | P263 : Avoid contact during pregnancy and while nursing. P260 : Do not breathe dust. P202 : Do not handle until all safety precautions have been read and understood. P201 : Obtain special instructions before use. P264 : Wash skin thoroughly after handling. P280 : Wear protective gloves/ protective clothing/ eye protection/ face protection/ hearing protection. |
Related Laws:
| RTECS# | GV0766666 |
Transport Information:
| HS Number | 2914790090 |
Application
Nitisinone: A Competitive Inhibitor of 4-Hydroxyphenyl-Pyruvate Dioxygenase (HPPD)
Nitisinone (NTBC) is a competitive inhibitor of 4-hydroxyphenyl-pyruvate dioxygenase (HPPD) which is an enzyme upstream of fumarylacetoacetate hydrolase (FAH) in the tyrosine catabolic pathway. By inhibiting HPPD, nitisinone prevents the production and accumulation of the catabolic intermediate metabolites of tyrosine, such as maleylacetoacetate fumarylacetoacetate. In clinical practice, patients with hereditary tyrosinemia type 1 (HT-1) who are deficient in fumarylacetoacetate hydrolase (FAH) required for the metabolism of the catabolic intermediate metabolites, have been reported to produce the toxic metabolites succinylacetone and succinylacetoacetate from these catabolic intermediate metabolites. Therefore, in HT-1 patients, nitisinone would suppresses tissue damage caused by the toxic metabolites. (The product is for research purpose only.)
References
- Treatment of hereditary tyrosinaemia type I by inhibition of 4-hydroxyphenylpyruvate dioxygenase
- From toxicological problem to therapeutic use: The discovery of the mode of action of 2-(2-nitro-4-trifluoromethylbenzoyl)-1,3-cyclohexanedione (NTBC), its toxicology and development as a drug (a review)
- Comparison of plasma and dry blood spots as samples for the determination of nitisinone (NTBC) by high-performance liquid chromatography-tandem mass spectrometry. Study of the stability of the samples at different temperatures
Product Documents (Note: Some products will not have analytical charts available.)
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