Nitisinone-13C6 is a stable isotope-labeled form of nitisinone, a pharmaceutical compound primarily used in the treatment of hereditary tyrosinemia type 1, a rare genetic disorder affecting the metabolism of the amino acid tyrosine. The "13C6" designation indicates that this compound contains six carbon atoms that are isotopically labeled with carbon-13, a non-radioactive isotope of carbon. This labeling is often utilized in research and clinical studies to trace metabolic pathways and understand the pharmacokinetics of the drug. Nitisinone functions as a competitive inhibitor of the enzyme 4-hydroxyphenylpyruvate dioxygenase, thereby reducing the accumulation of toxic metabolites associated with tyrosinemia. The compound is typically administered orally and has been shown to improve clinical outcomes in affected patients. Its chemical structure includes a pyrimidine ring and a hydroxyl group, contributing to its biological activity. As with any pharmaceutical, safety and efficacy are evaluated through clinical trials, and it is essential to monitor for potential side effects during treatment.

Nitisinone-13C6

1246815-63-3: Nitisinone-13C6 is a stable isotope-labeled form of nitisinone, a pharmaceutical compound primarily used in the treatment of hereditary tyrosinemia type 1, a rare genetic disorder affecting the metabolism of the amino acid tyrosine. The "13C6" designation indicates that this compound contains six carbon atoms that are isotopically labeled with carbon-13, a non-radioactive isotope of carbon. This labeling is often utilized in research and clinical studies to trace metabolic pathways and understand the pharmacokinetics of the drug. Nitisinone functions as a competitive inhibitor of the enzyme 4-hydroxyphenylpyruvate dioxygenase, thereby reducing the accumulation of toxic metabolites associated with tyrosinemia. The compound is typically administered orally and has been shown to improve clinical outcomes in affected patients. Its chemical structure includes a pyrimidine ring and a hydroxyl group, contributing to its biological activity. As with any pharmaceutical, safety and efficacy are evaluated through clinical trials, and it is essential to monitor for potential side effects during treatment.

<p>Applications Labelled Nitisinone. Nitisinone is a herbicidal triketone that inhibits 4-hydroxyphenylpyruvate dioxygenase (HPPD), an enzyme involved in plastoquinone biosynthesis in plants and in tyrosine catabolism in mammals. It is used in treatment of inherited tyrosinemia type I.<br>References Lindstedt, S., et al.: Lancet, 340, 813 (1992), Ellis, M.K., et al.: Toxicol. Appl. Pharmacol., 133, 12 (1995), Lock, E.A., et al.: J. Inherited Metab. Dis., 21, 498 (1998),<br></p>

CAS 1246815-63-3

Nitisinone-13C6

Nitisinone-13C6

Ref: TR-N490137