Nitisinone
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MedKoo CAT#: 314265

CAS#: 104206-65-7

Description: Nitisinone, also known as NTBC, is an effective herbicide, is the licensed treatment for the human condition, hereditary tyrosinaemia type 1 (HT-1). Its mode of action interrupts tyrosine metabolism through inhibition of 4-hydroxyphenylpyruvate dioxygenase (HPPD). Nitisinone is a remarkable safe drug to use with few side effects reported.


Price and Availability

Size Price Shipping out time Quantity
10mg USD 70 2 Weeks
25mg USD 90 2 Weeks
50mg USD 120 2 Weeks
100mg USD 150 2 Weeks
200mg USD 190 2 Weeks
500mg USD 350 2 Weeks
1g USD 450 2 Weeks
2g USD 650 2 Weeks
5g USD 850 2 Weeks
10g USD 1250 2 Weeks
20g USD 1950 2 Weeks
50g USD 2950 2 Weeks
100g USD 4250 2 Weeks
200g USD 6950 2 Weeks
Inquire bulk and customized quantity

Pricing updated 2020-11-23. Prices are subject to change without notice.

Nitisinone, purity > 98%, is in stock. Current shipping out time is about 2 weeks after order is received. CoA, QC data and MSDS documents are available in one week after order is received.


Chemical Structure

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Theoretical Analysis

MedKoo Cat#: 314265
Name: Nitisinone
CAS#: 104206-65-7
Chemical Formula: C14H10F3NO5
Exact Mass: 329.05111
Molecular Weight: 329.23
Elemental Analysis: C, 51.07; H, 3.06; F, 17.31; N, 4.25; O, 24.30


Synonym: NTBC; Nitisinone; brand name: Orfadin.

IUPAC/Chemical Name: 2-(2-nitro-4-(trifluoromethyl)benzoyl)cyclohexane-1,3-dione

InChi Key: OUBCNLGXQFSTLU-UHFFFAOYSA-N

InChi Code: InChI=1S/C14H10F3NO5/c15-14(16,17)7-4-5-8(9(6-7)18(22)23)13(21)12-10(19)2-1-3-11(12)20/h4-6,12H,1-3H2

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


Technical Data

Appearance:
Solid powder

Purity:
>98% (or refer to the Certificate of Analysis)

Shipping Condition:
Shipped under ambient temperature as non-hazardous chemical. This product is stable enough for a few weeks during ordinary shipping and time spent in Customs.

Storage Condition:
Dry, dark and at 0 - 4 C for short term (days to weeks) or -20 C for long term (months to years).

Solubility:
Soluble in DMSO.

Shelf Life:
>2 years if stored properly

Drug Formulation:
This drug may be formulated in DMSO

Stock Solution Storage:
0 - 4 C for short term (days to weeks), or -20 C for long term (months).

HS Tariff Code:
2934.99.9001


References

1: Souri E, Lahiji FR, Nourhashemi T, Jalalizadeh H. A Stability Indicating HPLC Method for the Determination of Nitisinone in Capsules. Indian J Pharm Sci. 2015 May-Jun;77(3):348-51. PubMed PMID: 26180282; PubMed Central PMCID: PMC4502151.

2: Simoncelli M, Samson J, Bussières JF, Lacroix J, Dorais M, Battista R, Perreault S. Cost-Consequence Analysis of Nitisinone for Treatment of Tyrosinemia Type I. Can J Hosp Pharm. 2015 May-Jun;68(3):210-7. PubMed PMID: 26157182; PubMed Central PMCID: PMC4485508.

3: Mistry JB, Jackson DJ, Bukhari M, Taylor AM. Osteoarticular cells tolerate short-term exposure to nitisinone-implications in alkaptonuria. Clin Rheumatol. 2015 May 31. [Epub ahead of print] PubMed PMID: 26024586.

4: Keenan CM, Preston AJ, Sutherland H, Wilson PJ, Psarelli EE, Cox TF, Ranganath LR, Jarvis JC, Gallagher JA. Nitisinone Arrests but Does Not Reverse Ochronosis in Alkaptonuric Mice. JIMD Rep. 2015;24:45-50. doi: 10.1007/8904_2015_437. Epub 2015 May 5. PubMed PMID: 25940034; PubMed Central PMCID: PMC4582025.

5: Olsson B, Cox TF, Psarelli EE, Szamosi J, Hughes AT, Milan AM, Hall AK, Rovensky J, Ranganath LR. Relationship Between Serum Concentrations of Nitisinone and Its Effect on Homogentisic Acid and Tyrosine in Patients with Alkaptonuria. JIMD Rep. 2015;24:21-7. doi: 10.1007/8904_2015_412. Epub 2015 Mar 13. PubMed PMID: 25772318; PubMed Central PMCID: PMC4582028.

6: Gertsman I, Barshop BA, Panyard-Davis J, Gangoiti JA, Nyhan WL. Metabolic Effects of Increasing Doses of Nitisinone in the Treatment of Alkaptonuria. JIMD Rep. 2015;24:13-20. doi: 10.1007/8904_2014_403. Epub 2015 Feb 10. PubMed PMID: 25665838; PubMed Central PMCID: PMC4582031.

7: Hughes AT, Milan AM, Davison AS, Christensen P, Ross G, Gallagher JA, Dutton JJ, Ranganath LR. Serum markers in alkaptonuria: simultaneous analysis of homogentisic acid, tyrosine and nitisinone by liquid chromatography tandem mass spectrometry. Ann Clin Biochem. 2015 Sep;52(Pt 5):597-605. doi: 10.1177/0004563215571969. Epub 2015 Jan 27. PubMed PMID: 25628464.

8: Ranganath LR, Milan AM, Hughes AT, Dutton JJ, Fitzgerald R, Briggs MC, Bygott H, Psarelli EE, Cox TF, Gallagher JA, Jarvis JC, van Kan C, Hall AK, Laan D, Olsson B, Szamosi J, Rudebeck M, Kullenberg T, Cronlund A, Svensson L, Junestrand C, Ayoob H, Timmis OG, Sireau N, Le Quan Sang KH, Genovese F, Braconi D, Santucci A, Nemethova M, Zatkova A, McCaffrey J, Christensen P, Ross G, Imrich R, Rovensky J. Suitability Of Nitisinone In Alkaptonuria 1 (SONIA 1): an international, multicentre, randomised, open-label, no-treatment controlled, parallel-group, dose-response study to investigate the effect of once daily nitisinone on 24-h urinary homogentisic acid excretion in patients with alkaptonuria after 4 weeks of treatment. Ann Rheum Dis. 2014 Dec 4. pii: annrheumdis-2014-206033. doi: 10.1136/annrheumdis-2014-206033. [Epub ahead of print] PubMed PMID: 25475116.

9: Lock E, Ranganath LR, Timmis O. The role of nitisinone in tyrosine pathway disorders. Curr Rheumatol Rep. 2014 Nov;16(11):457. doi: 10.1007/s11926-014-0457-0. Review. PubMed PMID: 25266991.

10: Stewart RM, Briggs MC, Jarvis JC, Gallagher JA, Ranganath L. Reversible keratopathy due to hypertyrosinaemia following intermittent low-dose nitisinone in alkaptonuria: a case report. JIMD Rep. 2014;17:1-6. doi: 10.1007/8904_2014_307. Epub 2014 Jul 6. PubMed PMID: 24997710; PubMed Central PMCID: PMC4241204.

11: Bartlett DC, Lloyd C, McKiernan PJ, Newsome PN. Early nitisinone treatment reduces the need for liver transplantation in children with tyrosinaemia type 1 and improves post-transplant renal function. J Inherit Metab Dis. 2014 Sep;37(5):745-52. doi: 10.1007/s10545-014-9683-x. Epub 2014 Feb 11. PubMed PMID: 24515874.

12: Bendadi F, de Koning TJ, Visser G, Prinsen HC, de Sain MG, Verhoeven-Duif N, Sinnema G, van Spronsen FJ, van Hasselt PM. Impaired cognitive functioning in patients with tyrosinemia type I receiving nitisinone. J Pediatr. 2014 Feb;164(2):398-401. doi: 10.1016/j.jpeds.2013.10.001. Epub 2013 Nov 14. PubMed PMID: 24238861.

13: Preston AJ, Keenan CM, Sutherland H, Wilson PJ, Wlodarski B, Taylor AM, Williams DP, Ranganath LR, Gallagher JA, Jarvis JC. Ochronotic osteoarthropathy in a mouse model of alkaptonuria, and its inhibition by nitisinone. Ann Rheum Dis. 2014 Jan;73(1):284-9. doi: 10.1136/annrheumdis-2012-202878. Epub 2013 Mar 19. PubMed PMID: 23511227.

14: Vanclooster A, Devlieger R, Meersseman W, Spraul A, Kerckhove KV, Vermeersch P, Meulemans A, Allegaert K, Cassiman D. Pregnancy during nitisinone treatment for tyrosinaemia type I: first human experience. JIMD Rep. 2012;5:27-33. doi: 10.1007/8904_2011_88. Epub 2011 Dec 16. PubMed PMID: 23430914; PubMed Central PMCID: PMC3509920.

15: Wisse RP, Wittebol-Post D, Visser G, van der Lelij A. Corneal depositions in tyrosinaemia type I during treatment with Nitisinone. BMJ Case Rep. 2012 Nov 30;2012. pii: bcr2012006301. doi: 10.1136/bcr-2012-006301. PubMed PMID: 23203167; PubMed Central PMCID: PMC4543320.

16: Larochelle J, Alvarez F, Bussières JF, Chevalier I, Dallaire L, Dubois J, Faucher F, Fenyves D, Goodyer P, Grenier A, Holme E, Laframboise R, Lambert M, Lindstedt S, Maranda B, Melançon S, Merouani A, Mitchell J, Parizeault G, Pelletier L, Phan V, Rinaldo P, Scott CR, Scriver C, Mitchell GA. Effect of nitisinone (NTBC) treatment on the clinical course of hepatorenal tyrosinemia in Québec. Mol Genet Metab. 2012 Sep;107(1-2):49-54. doi: 10.1016/j.ymgme.2012.05.022. Epub 2012 Jul 13. PubMed PMID: 22885033.

17: Onojafe IF, Adams DR, Simeonov DR, Zhang J, Chan CC, Bernardini IM, Sergeev YV, Dolinska MB, Alur RP, Brilliant MH, Gahl WA, Brooks BP. Nitisinone improves eye and skin pigmentation defects in a mouse model of oculocutaneous albinism. J Clin Invest. 2011 Oct;121(10):3914-23. doi: 10.1172/JCI59372. PubMed PMID: 21968110; PubMed Central PMCID: PMC3223618.

18: Manga P, Orlow SJ. Informed reasoning: repositioning of nitisinone to treat oculocutaneous albinism. J Clin Invest. 2011 Oct;121(10):3828-31. doi: 10.1172/JCI59763. PubMed PMID: 21968107; PubMed Central PMCID: PMC3195484.

19: Introne WJ, Perry MB, Troendle J, Tsilou E, Kayser MA, Suwannarat P, O'Brien KE, Bryant J, Sachdev V, Reynolds JC, Moylan E, Bernardini I, Gahl WA. A 3-year randomized therapeutic trial of nitisinone in alkaptonuria. Mol Genet Metab. 2011 Aug;103(4):307-14. doi: 10.1016/j.ymgme.2011.04.016. Epub 2011 May 6. PubMed PMID: 21620748; PubMed Central PMCID: PMC3148330.

20: Prieto JA, Andrade F, Lage S, Aldámiz-Echevarría L. 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. J Chromatogr B Analyt Technol Biomed Life Sci. 2011 Apr 1;879(11-12):671-6. doi: 10.1016/j.jchromb.2011.01.031. Epub 2011 Feb 2. PubMed PMID: 21377430.