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Nitisinone-13C6

CAT: 0931-T36055Size: 1 mgDry Ice: NoHazardous: No
CAT#:0931-T36055Size:1 mg
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24/48H Stock Items & 2 to 6 Weeks non Stock Items.
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CAS Number
1246815-63-3
Bioactivity
Nitisinone-13C6is intended for use as an internal standard for the quantification of nitisinone by GC- or LC-MS. Nitisinone is an inhibitor of 4-hydroxyphenylpyruvate dioxygenase (HPPD), which converts 4-hydroxyphenylpyruvate (HPPA) to homogentisate in the tyrosine catabolic pathway.1Nitisinone 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.2It exhibits hepatoprotective effects inFAH-/-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 ?g) decreases urinary excretion of homogentisate and increases urinary excretion of HPPA, HPLA, and 4-hydroxyphenylacetate in a mouse model of alkaptonuria induced by ethylnitrosourea.3Formulations containing nitisinone have been used in the treatment of hereditary tyrosinemia type 1 (HT-1) .
Smiles
FC(F)(F)c1ccc(C(=O)[13CH]2[13C](=O)[13CH2][13CH2][13CH2][13C]2=O)c(c1)N(=O)=O
Molecular Formula
C14H10F3NO5
Molecular Weight
335.185
Shipping Conditions
Cool pack
Storage Temperature
-20°C

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