Name: Nitisinone-13C6
Rating: 5 (10 reviews)

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HIT ID:HIT214320947
Cas No:1246815-63-3
常用名:Nitisinone-13C6
别名:Nitisinone-13C6
IUPAC:2-[2-nitro-4-(trifluoromethyl)benzoyl](1,2,3,4,5,6-13C6)cyclohexane-1,3-dione
IUPAC:2-[2-nitro-4-(trifluoromethyl)benzoyl](1,2,3,4,5,6-13C6)cyclohexane-1,3-dione
InChI key:OUBCNLGXQFSTLU-GRIYWAASSA-N

分子式:C14H10F3NO5
分子量:335.185
氢键供体数 (HBD):
氢键受体数 (HBA):
LogP:
SMILES:FC(F)(F)c1ccc(C(=O)[13CH]2[13C](=O)[13CH2][13CH2][13CH2][13C]2=O)c(c1)N(=O)=O
SMILES:FC(F)(F)c1ccc(C(=O)[13CH]2[13C](=O)[13CH2][13CH2][13CH2][13C]2=O)c(c1)N(=O)=O

Nitisinone-13C6is intended for use as an internal standard for the quantification of nitisinone by GC- or LC-MS. Nitisinone is an inhibitor of 4-hydroxyphenylpyruvate dioxygenase (HPPD), which converts 4-hydroxyphenylpyruvate (HPPA) to homogentisate in the tyrosine catabolic pathway.1Nitisinone increases urinary levels of HPPA and 4-hydroxyphenyllactate (HPLA) in rats when administered at a dose of 10 mg/kg. Nitisinone (3 mg/kg) prevents the neonatal lethality of fumarylacetoacetate hydrolase (FAH) deficiency in mice when administered to pregnant dams.2It exhibits hepatoprotective effects inFAH-/-mice, such as prevention of increases in plasma levels of aspartate serine aminotransferase (AST) and conjugated bilirubin, when administration is continued following birth at a dose of 1 mg/kg. Nitisinone (100 μg) decreases urinary excretion of homogentisate and increases urinary excretion of HPPA, HPLA, and 4-hydroxyphenylacetate in a mouse model of alkaptonuria induced by ethylnitrosourea.3Formulations containing nitisinone have been used in the treatment of hereditary tyrosinemia type 1 (HT-1).

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