Nitisinone-13C6

Nitisinone-13C6 is intended for use as an internal standard for the quantification of nitisinone (Cay-17924) 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. Nitisinone 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. It exhibits hepatoprotective effects in FAH-/- 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. Formulations containing nitisinone have been used in the treatment of hereditary tyrosinemia type 1 (HT-1).Formal Name: 2-(2-nitro-4-(trifluoromethyl)benzoyl)cyclohexane-1,3-dione-1,2,3,4,5,6-13C6. CAS Number: 1246815-63-3. Synonyms: NTBC-13C6. Molecular Formula: C8[13C6]H10F3NO5. Formula Weight: 335.2. Purity: >98%. Formulation: (Request formulation change), A solid. Solubility: Chloroform: slightly soluble, Ethyl Acetate: slightly soluble. SMILES: O=[13C]([13CH2][13CH2][13CH2]1)[13CH](C(C2=CC=C(C(F)(F)F)C=C2[N+]([O-])=O)=O)[13C]1=O. 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/i1+1,2+1,3+1,10+1,11+1,12+1. InChi Key: OUBCNLGXQFSTLU-GRIYWAASSA-N.