Shelf life (days): 1460.0
Formulation: A solid
Formal Name: 2-(2-nitro-4-(trifluoromethyl)benzoyl)cyclohexane-1,3-dione-1,2,3,4,5,6-13C6
Purity: ≥98%
Formula Markup: C8[13C6]H10F3NO5
Formula Weight: 335.2
CAS Number: 1246815-63-3
Notes: Nitisinone-13C6 is intended for use as an internal standard for the quantification of nitisinone (Item No. 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.{42952} 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.{42953} 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.{42954} Formulations containing nitisinone have been used in the treatment of hereditary tyrosinemia type 1 (HT-1).
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