Shelf life (days): 1460.0
Formulation: A crystalline solid
Formal Name: 2-[2-nitro-4-(trifluoromethyl)benzoyl]-1,3-cyclohexanedione
Purity: ≥98%
Formula Markup: C14H10F3NO5
Formula Weight: 329.2
CAS Number: 104206-65-7
Notes: 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).
Deutsch
English
