Date published: 2025-12-21

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Nitisinone (CAS 104206-65-7)

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Alternate Names:
2-(2-Nitro-4-trifluoromethylbenzoyl)-1,3-cyclohexanedione; 2-[2-Nitro-4-(trifluoromethyl)benzoyl]cyclohexane-1,3-dione
Application:
Nitisinone is an herbicidil triketone that inhibits 4-hydroxyphenylpyruvate dioxygenase (HPPD)
CAS Number:
104206-65-7
Purity:
≥97%
Molecular Weight:
329.23
Molecular Formula:
C14H10F3NO5
For Research Use Only. Not Intended for Diagnostic or Therapeutic Use.
* Refer to Certificate of Analysis for lot specific data.

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Nitisinone is a potent inhibitor of 4-hydroxyphenylpyruvate dioxygenase (HPPD), an enzyme involved in the catabolism of tyrosine. By inhibiting HPPD, nitisinone effectively blocks the conversion of 4-hydroxyphenylpyruvate to homogentisic acid, leading to the accumulation of 4-hydroxyphenylpyruvate and its metabolites. Nitisinone′s function as an HPPD inhibitor allows the study about the biochemical pathways involved in tyrosine metabolism and the potential implications of modulating these pathways.


Nitisinone (CAS 104206-65-7) References

  1. Nitisinone in the treatment of hereditary tyrosinaemia type 1.  |  McKiernan, PJ. 2006. Drugs. 66: 743-50. PMID: 16706549
  2. Early nitisinone treatment reduces the need for liver transplantation in children with tyrosinaemia type 1 and improves post-transplant renal function.  |  Bartlett, DC., et al. 2014. J Inherit Metab Dis. 37: 745-52. PMID: 24515874
  3. Cost-Consequence Analysis of Nitisinone for Treatment of Tyrosinemia Type I.  |  Simoncelli, M., et al. 2015. Can J Hosp Pharm. 68: 210-7. PMID: 26157182
  4. Nitisinone causes acquired tyrosinosis in alkaptonuria.  |  Khedr, M., et al. 2020. J Inherit Metab Dis. 43: 1014-1023. PMID: 32083330
  5. Inter-laboratory analytical improvement of succinylacetone and nitisinone quantification from dried blood spot samples.  |  Laeremans, H., et al. 2020. JIMD Rep. 53: 90-102. PMID: 32395414
  6. Influence of nitisinone and its metabolites on l-tyrosine metabolism in a model system.  |  Płonka, J., et al. 2022. Chemosphere. 286: 131592. PMID: 34311397
  7. Preventive use of nitisinone in alkaptonuria.  |  Wolffenbuttel, BHR., et al. 2021. Orphanet J Rare Dis. 16: 343. PMID: 34344451
  8. Nitisinone treatment during two pregnancies and breastfeeding in a woman with tyrosinemia type 1 - a case report.  |  Zöggeler, T., et al. 2022. J Pediatr Endocrinol Metab. 35: 259-265. PMID: 34506697
  9. Comparing nitisinone 2 mg and 10 mg in the treatment of alkaptonuria-An approach using statistical modelling.  |  Ranganath, LR., et al. 2022. JIMD Rep. 63: 80-92. PMID: 35028273
  10. Characterization of changes in the tyrosine pathway by 24-h profiling during nitisinone treatment in alkaptonuria.  |  Ranganath, LR., et al. 2022. Mol Genet Metab Rep. 30: 100846. PMID: 35242577
  11. Long-term low dose nitisinone therapy in adults with alkaptonuria shows no cognitive decline or increased severity of depression.  |  Davison, AS., et al. 2022. JIMD Rep. 63: 221-230. PMID: 35433173
  12. Impact of Nitisinone on the Cerebrospinal Fluid Metabolome of a Murine Model of Alkaptonuria.  |  Davison, AS., et al. 2022. Metabolites. 12: PMID: 35736410
  13. Adequacy of nitisinone for the management of alkaptonuria.  |  Abbas, K., et al. 2022. Ann Med Surg (Lond). 80: 104340. PMID: 36045846
  14. Determinants of tyrosinaemia during nitisinone therapy in alkaptonuria.  |  Ranganath, LR., et al. 2022. Sci Rep. 12: 16083. PMID: 36167967
  15. Comprehensive Biotransformation Analysis of Phenylalanine-Tyrosine Metabolism Reveals Alternative Routes of Metabolite Clearance in Nitisinone-Treated Alkaptonuria.  |  Norman, BP., et al. 2022. Metabolites. 12: PMID: 36295829

Ordering Information

Product NameCatalog #UNITPriceQtyFAVORITES

Nitisinone, 10 mg

sc-208100
10 mg
$98.00