Description
An inhibitor of HPPD; increases urinary levels of HPPA and HPLA in rats at 10 mg/kg; prevents the neonatal lethality of FAH deficiency in mice when administered to pregnant dams at 3 mg/kg; exhibits hepatoprotective effects in FAH-/- mice, such as prevention of increases in plasma levels of AST and conjugated bilirubin, when administration is continued following birth at 1 mg/kg; decreases urinary excretion of homogentisate and increases urinary excretion of HPPA, HPLA, and 4-hydroxyphenylacetate in a mouse model of ethylnitrosourea-induced alkaptonuria at 100 µg