104206-65-7

Nitisinone (SC0735)(SC0735) is an enzyme 4-hydroxyphenylpyruvate dioxygenase inhibitor.

Nitisinone-13C6 is intended for use as an internal standard for the quantification of nitisinone by GC- or LC-MS. Nitisinone (T1684) 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 (T1684) (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 (T1684) (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).

Nitisinone-13C6 (Standard) is a reference standard of Nitisinone-13C6 intended for quantitative analysis, quality control, and related biochemical research applications. Nitisinone-13C6 is used as an internal standard when quantitatively analyzing nitisinone by gas chromatography-mass spectrometry or liquid chromatography-mass spectrometry. Nitisinone is an inhibitor of 4-hydroxyphenylpyruvate dioxygenase (HPPD), which converts 4-hydroxyphenylpyruvate (HPPA) to homovanillic acid during the tyrosine degradation pathway.