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

Nitisinone – 250 mg

Brand:
Cayman
CAS:
104206-65-7
Storage:
-20
UN-No:
De Minimis - 2811 / 6.1

Nitisinone is an inhibitor of 4-hydroxyphenylpyruvate dioxygenase (HPPD), which converts 4-hydroxyphenylpyruvate (HPPA) to homogentisate in the tyrosine catabolic pathway.{42952} Nitisinone increases urinary levels of HPPA and 4-hydroxyphenyllactate (HPLA) in rats when administered at a dose of 10 mg/kg. Nitisinone (3 mg/kg) prevents the neonatal lethality of fumarylacetoacetate hydrolase (FAH) deficiency in mice when administered to pregnant dams.{42953} It exhibits hepatoprotective effects in FAH-/- 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 (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.{42954} Formulations containing nitisinone have been used in the treatment of hereditary tyrosinemia type 1 (HT-1).  

 

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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

Formal name: 2-[2-nitro-4-(trifluoromethyl)benzoyl]-1,3-cyclohexanedione

Synonyms:  NTBC|SC-0735

Molecular weight: 329.2

CAS: 104206-65-7

Purity: ≥98%

Formulation: A crystalline solid

Product Type|Biochemicals|Small Molecule Inhibitors|Amino Acid Turnover||Research Area|Endocrinology & Metabolism|Inborn Errors of Metabolism||Research Area|Plant Biology|Phytotoxins

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