Nitrisinone

Nitirisinone is a chemical compound originally developed as an herbicide, but now being investigated as a treatment for certain forms of type 1 tyrosinaemia. The mechanism of action of nitrisinone involves inhibition of the enzyme 4-hydroxyphenylpyruvic acid oxidase, thus preventing the formation of maleylacetoacetic acid and fumarylacetoacetic acid, which have the potential to be converted to succinyl acetone, a toxin that damages the liver and kidneys.