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Nitisinone

$360

  • Brand : BIOFRON

  • Catalogue Number : BN-O1443

  • Specification : 98%(HPLC)

  • CAS number : 104206-65-7

  • Formula : C14H10F3NO5

  • Molecular Weight : 329.23

  • PUBCHEM ID : 115355

  • Volume : 20mg

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Catalogue Number

BN-O1443

Analysis Method

Specification

98%(HPLC)

Storage

2-8°C

Molecular Weight

329.23

Appearance

Botanical Source

Structure Type

Category

SMILES

C1CC(=O)C(C(=O)C1)C(=O)C2=C(C=C(C=C2)C(F)(F)F)[N+](=O)[O-]

Synonyms

nitisinonum/2-(2-Nitro-4-trifluoromethylbenzoyl)-1,3-cyclohexanedione/2-[2-Nitro-4-(trifluoromethyl)benzoyl]-1,3-cyclohexanedione/2-(2-nitro-4-(trifluoromethyl)benzoyl)cyclohexane-1,3-dione/Orfadin/Nitisinone [INN]/nitisinona/Orfadin (TN)/1,3-Cyclohexanedione, 2-[2-nitro-4-(trifluoromethyl)benzoyl]-/Nitisone/2-(2-Nitro-4-trifluoromethylbenzoyl)cyclohexane-1,3-dione/2-[2-nitro-4-(trifluoromethyl)benzoyl]cyclohexane-1,3-dione/Nitisinone

IUPAC Name

2-[2-nitro-4-(trifluoromethyl)benzoyl]cyclohexane-1,3-dione

Density

1.5±0.1 g/cm3

Solubility

Flash Point

247.9±28.7 °C

Boiling Point

486.2±45.0 °C at 760 mmHg

Melting Point

InChl

InChl Key

OUBCNLGXQFSTLU-UHFFFAOYSA-N

WGK Germany

RID/ADR

HS Code Reference

Personal Projective Equipment

Correct Usage

For Reference Standard and R&D, Not for Human Use Directly.

Meta Tag

provides coniferyl ferulate(CAS#:104206-65-7) MSDS, density, melting point, boiling point, structure, formula, molecular weight etc. Articles of coniferyl ferulate are included as well.>> amp version: coniferyl ferulate

No Technical Documents Available For This Product.

PMID

31901053

Abstract

OBJECTIVE:
To analyze the results of screening for hereditary tyrosinemia (HT) in newborns and its clinical features and genotype.

METHODS:
The HT screening was conducted among 2 188 784 newborns from November 2013 to November 2018. The tyrosine (TYR)/ succinylacetone (SA) levels were detected by tandem mass spectrometry (MS-MS). The clinical characteristics, genetic results and following up data of identified patients were analyzed.

RESULTS:
The normal ranges (0.5%-95.5%) of TYR and SA were 34.5-280.0 μmol/L and 0.16-2.58 μmol/L, respectively. Three HT cases were confirmed with a detection rate of 1∶729 595. There was 1 case of tyrosinemia type Ⅰ (HTⅠ) (homozygous variations of c.455G>A in FAH gene), 1 case of tyrosinemia type Ⅱ(HTⅡ) (heterozygous variations of c.890G>T and c.408+1G>A in TAT gene), and 1 case of tyrosinemia type Ⅲ (HT Ⅲ) (homozygous variations of c.257T>C in HPD gene). The variations of c.890G>T, c.4081G>A of TAT and c.257T>C of HPD were novel. The positive predictive value of the screening was 3.4%. Case 1 (HTⅠ) with TYR and SA values of 666.9 μmol/L and 3.87 μmol/L respectively, presented cholestasis, mild elevated of liver enzyme and lactic acid, who were although fed with TYR and phenylalanine free milk, but died at 2 months of age. Case 2 (HTⅡ) with higher TYR (625.6 μmol/L) and normal SA at screening, received medical milk treatment; during the 7 months of follow-up the baby showed normal score of Bayley assessment and normal TYR without eye and skin symptoms. Case 3 (HT Ⅲ) with TYR of 1035.3 μmol/L and normal SA at screening; during the 29 months of follow-up the value of TYR fluctuated from 532.1 μmol/L to 1060.3 μmol/L due to irregular medical milk treatment, while the score of Bayley assessment was normal.

CONCLUSIONS:
HT is rare in the southern Chinese population, and the gene spectrum is scattered. Early treatment with nitisinone is recommended in children with HTⅠ, otherwise the prognosis is poor; the prognosis of children with HTⅡ is good when early treated with special diet; the prognosis of children with HTⅢ needs to be determined with more data.

Title

[Screening for hereditary tyrosinemia and genotype analysis in newborns].

Author

Tong F1, Yang R1, Liu C1, Wu D1, Zhang T1, Huang X1, Hong F1, Qian G1, Huang X1, Zhou X1, Shu Q1, Zhao Z1.

Publish date

2019 Jun 25

PMID

31667718

Abstract

Tyrosinemia type 1 (TT1) is a rare metabolic disease caused by a defect in tyrosine catabolism. TT1 is clinically characterized by acute liver failure, development of hepatocellular carcinoma, renal and neurological problems, and consequently an extremely poor outcome. This review showed that the introduction of 2-(2-nitro-4-trifluoromethylbenzoyl)-1,3-cyclohexanedione (NTBC) in 1992 has revolutionized the outcome of TT1 patients, especially when started pre-clinically. If started early, NTBC can prevent liver failure, renal problems, and neurological attacks and decrease the risk for hepatocellular carcinoma. NTBC has been shown to be safe and well tolerated, although the long-term effectiveness of treatment with NTBC needs to be awaited. The high tyrosine concentrations caused by treatment with NTBC could result in ophthalmological and skin problems and requires life-long dietary restriction of tyrosine and its precursor phenylalanine, which could be strenuous to adhere to. In addition, neurocognitive problems have been reported since the introduction of NTBC, with hypothesized but as yet unproven pathophysiological mechanisms. Further research should be done to investigate the possible relationship between important clinical outcomes and blood concentrations of biochemical parameters such as phenylalanine, tyrosine, succinylacetone, and NTBC, and to develop clear guidelines for treatment and follow-up with reliable measurements. This all in order to ultimately improve the combined NTBC and dietary treatment and limit possible complications such as hepatocellular carcinoma development, neurocognitive problems, and impaired quality of life.

Title

Long-Term Outcomes and Practical Considerations in the Pharmacological Management of Tyrosinemia Type 1.

Author

van Ginkel WG1, Rodenburg IL2, Harding CO3, Hollak CEM4, Heiner-Fokkema MR5, van Spronsen FJ6.

Publish date

2019 Dec

PMID

31623189

Abstract

Tyrosinemia type 1 (TT1) is a rare metabolic disease caused by a defect in the tyrosine degradation pathway. Neurocognitive deficiencies have been described in TT1 patients, that have, among others, been related to changes in plasma large neutral amino acids (LNAA) that could result in changes in brain LNAA and neurotransmitter concentrations. Therefore, this project aimed to investigate plasma and brain LNAA, brain neurotransmitter concentrations and behavior in C57 Bl/6 fumarylacetoacetate hydrolase deficient (FAH-/-) mice treated with 2-(2-nitro-4-trifluoromethylbenoyl)-1,3-cyclohexanedione (NTBC) and/or diet and wild-type mice. Plasma and brain tyrosine concentrations were clearly increased in all NTBC treated animals, even with diet (p < 0.001). Plasma and brain phenylalanine concentrations tended to be lower in all FAH-/- mice. Other brain LNAA, were often slightly lower in NTBC treated FAH-/- mice. Brain neurotransmitter concentrations were usually within a normal range, although serotonin was negatively correlated with brain tyrosine concentrations (p < 0.001). No clear behavioral differences between the different groups of mice could be found. To conclude, this is the first study measuring plasma and brain biochemistry in FAH-/- mice. Clear changes in plasma and brain LNAA have been shown. Further research should be done to relate the biochemical changes to neurocognitive impairments in TT1 patients.

KEYWORDS

FAH; NTBC; brain biochemistry; large neutral amino acids; mice; phenylalanine; tyrosine; tyrosinemia type 1

Title

Blood and Brain Biochemistry and Behaviour in NTBC and Dietary Treated Tyrosinemia Type 1 Mice.

Author

van Ginkel WG1, van Vliet D2, van der Goot E3, Faassen MHJR4, Vogel A5, Heiner-Fokkema MR6, van der Zee EA7, van Spronsen FJ8.

Publish date

2019 Oct 16


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