to treat Ixodes scapularis tick cells to inhibit the activity of hydroxyphenylpyruvate dioxygenase[2]
in supplemented water to block the accumulation of toxic metabolites in human hepatocyte engrafted mice[3]
to study its effect on bacterial pyomelanin production[4]
Biochem/physiol Actions
Nitisinone is a competitive and reversible inhibitor of 4-Hydroxyphenylpyruvate oxidase (dioxygenase).
Nitisinone is a competitive inhibitor that reversibly inhibits 4-Hydroxyphenylpyruvate oxidase (dioxygenase). Nitisinone is used in the treatment of hereditary tyrosinemia type 1, where it blocks the degradation of tyrosine into harmful substances.
Annals of the rheumatic diseases, 73(1), 284-289 (2013-03-21)
Alkaptonuria (AKU) is a rare metabolic disease caused by deficiency of homogentisate 1,2 dioxygenase, an enzyme involved in tyrosine catabolism, resulting in increased circulating homogentisic acid (HGA). Over time HGA is progressively deposited as a polymer (termed ochronotic pigment) in
To describe the confocal microscopic findings in a patient with hereditary tyrosinemia type I (HT-I) treated with 2-(2-nitro-4-trifluoromethylbenzoyl)-1,3-cyclohexanedione (NTBC) who developed corneal crystals. In this case study, we describe the confocal microscopic findings in a boy, who was diagnosed with
Molecular genetics and metabolism, 107(1-2), 49-54 (2012-08-14)
Hepatorenal tyrosinemia (HT1, fumarylacetoacetate hydrolase deficiency, MIM 276700) can cause severe hepatic, renal and peripheral nerve damage. In Québec, HT1 is frequent and neonatal HT1 screening is practiced. Nitisinone (NTBC, Orfadin ®) inhibits tyrosine degradation prior to the formation of
Molecular genetics and metabolism, 102(2), 122-125 (2010-11-30)
Psychomotor impairment has been described in hypertyrosinemia types II and III (HT III). Only recently cognitive deficits have also been reported in hypertyrosinemia type I (HT I). The pathogenic mechanisms responsible are unknown. Since implementation of 2-(2-nitro-4-trifluoromethylbenzoyl)-1,3-cyclohexanedione (NTBC, Nitisinone (Swedish
Immunization with AgTRIO, a protein in Anopheles saliva, contributes to protection against Plasmodium infection in mice
Our team of scientists has experience in all areas of research including Life Science, Material Science, Chemical Synthesis, Chromatography, Analytical and many others.
