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Clinical trials are ongoing to test whether nitisinone can prevent ochronosis experienced by older alkaptonuria patients. History
Nitisinone was discovered as part of a program to develop a class of herbicides called HPPD inhibitors. It is a member of the benzoylcyclohexane-1,3-dione family of herbicides, which are chemically derived from a natural phytotoxin, leptospermone, obtained from the Australian bottlebrush plant (Callistemon citrinus). HPPD is essential in plants and animals for catabolism, or breaking apart, of tyrosine. In plants, preventing this process leads to destruction of chlorophyll and the death of the plant. In toxicology studies of the herbicide, it was discovered that it had activity against HPPD in rats and humans.In Type I tyrosinemia, a different enzyme involved in the breakdown of tyrosine, fumarylacetoacetate hydrolase is mutated and doesnt work, leading to very harmful products building up in the body. Fumarylacetoacetate hydrolase acts on tyrosine after HPPD does, so scientists working on making herbicides in the class of HPPD inhibitors hypothesized that inhibiting HPPD and controlling tyrosine in the diet could treat this disease. A series of small clinical trials attempted with one of their compounds, nitisinone, were conducted and were successful, leading to nitisinone being brought to market as an orphan drug  Swedish Orphan International, which was later acquired by Swedish Orphan Biovitrum (Sobi). In 201, Cycle Pharmaceuticals launched Nityr in the United States through Cycle Vita. Research
Nitisinone is being studied as a treatment for alkaptonuria. References
External links
"Nitisinone". Drug Information Portal. U.S. National Library of Medicine.