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SML0269

Sigma-Aldrich

Nitisinone

≥95% (HPLC)

Synonym(s):

2-(2-Nitro-4-trifluoromethylbenzoyl)-1,3-cyclohexanedione, 2-[2-Nitro-4-(trifluoromethyl)benzoyl]cyclohexane-1,3-dione, NTBC, Nitisone, SC 0735

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About This Item

Empirical Formula (Hill Notation):
C14H10F3NO5
CAS Number:
104206-65-7
Molecular Weight:
329.23
MDL number:
MFCD01752192
UNSPSC Code:
12352200
PubChem Substance ID:
329825277
NACRES:
NA.77

Quality Level

100

Assay

≥95% (HPLC)

form

powder

color

white to brown

solubility

DMSO: ≥5 mg/mL

storage temp.

−20°C

SMILES string

[O-][N+](=O)c1cc(ccc1C(=O)C2C(=O)CCCC2=O)C(F)(F)F

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

InChI key

OUBCNLGXQFSTLU-UHFFFAOYSA-N

Gene Information

human ... HPD(3242)

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Application

Nitisinone has been used:
  • to induce liver injury
  • to treat Ixodes scapularis tick cells to inhibit the activity of hydroxyphenylpyruvate dioxygenase
  • in supplemented water to block the accumulation of toxic metabolites in human hepatocyte engrafted mice
  • to study its effect on bacterial pyomelanin production

Biochem/physiol Actions

Nitisinone is a competitive inhibitor that reversibly inhibits 4-Hydroxyphenylpyruvate oxidase (dioxygenase). Nitisinone is used in the treatment of hereditary tyrosinemia type 1, where it blocks the degradation of tyrosine into harmful substances.

Storage Class Code

11 - Combustible Solids

WGK

WGK 3

Flash Point(F)

Not applicable

Flash Point(C)

Not applicable

Certificates of Analysis (COA)

Search for Certificates of Analysis (COA) by entering the products Lot/Batch Number. Lot and Batch Numbers can be found on a product’s label following the words ‘Lot’ or ‘Batch’.

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Jean Larochelle et al.
Molecular genetics and metabolism, 107(1-2), 49-54 (2012-08-14)
Hepatorenal tyrosinemia (HT1, fumarylacetoacetate hydrolase deficiency, MIM 276700) can cause severe hepatic, renal and peripheral nerve damage. In Québec, HT1 is frequent and neonatal HT1 screening is practiced. Nitisinone (NTBC, Orfadin ®) inhibits tyrosine degradation prior to the formation of
Pieter-Paul Schauwvlieghe et al.
Cornea, 32(1), 91-94 (2012-04-13)
To describe the confocal microscopic findings in a patient with hereditary tyrosinemia type I (HT-I) treated with 2-(2-nitro-4-trifluoromethylbenzoyl)-1,3-cyclohexanedione (NTBC) who developed corneal crystals. In this case study, we describe the confocal microscopic findings in a boy, who was diagnosed with
Andrew J Preston et al.
Annals of the rheumatic diseases, 73(1), 284-289 (2013-03-21)
Alkaptonuria (AKU) is a rare metabolic disease caused by deficiency of homogentisate 1,2 dioxygenase, an enzyme involved in tyrosine catabolism, resulting in increased circulating homogentisic acid (HGA). Over time HGA is progressively deposited as a polymer (termed ochronotic pigment) in
Methods to inhibit bacterial pyomelanin production and determine the corresponding increase in sensitivity to oxidative stress
Ketelboeter LM and Bardy SL
Journal of Visualized Experiments, (102), e53105-e53105 (2015)
Eva Thimm et al.
Molecular genetics and metabolism, 102(2), 122-125 (2010-11-30)
Psychomotor impairment has been described in hypertyrosinemia types II and III (HT III). Only recently cognitive deficits have also been reported in hypertyrosinemia type I (HT I). The pathogenic mechanisms responsible are unknown. Since implementation of 2-(2-nitro-4-trifluoromethylbenzoyl)-1,3-cyclohexanedione (NTBC, Nitisinone (Swedish
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