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1

Inhibition of 4-hydroxyphenylpyruvate dioxygenase by 2-[2-nitro-4-[trifluoromethyl]benzoyl]-1,3-cyclohexanedione

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Molchanov S., Gryff-Keller A.
Acta Biochimica Polonica
|
2009
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tom 56
|
nr 3
447-454
Triketone herbicides are inhibitors of 4-hydroxyphenylpyruvate dioxygenase (HPPD), a key enzyme of the tyrosine transformation pathway, common for plants and animals. One of these herbicides, 2-[2-nitro-4-(trifluoromethyl)benzoyl]-1,3-cyclohexanedione (NTBC), is so selective and efficient that it can be applied as a medicine in a hereditary metabolic disease - tyrosinemia type I. In this paper the available information concerning the molecular mechanism of HPPD inhibition by NTBC, originating from experimental investigations as well as theoretical modeling, has been collected. It is supplemented by results of additional theoretical DFT and/or MP2 calculations of the energetic effects of individual elementary molecular transformations. All these data are discussed and a consistent picture of HPPD inhibition by NTBC is proposed.
2

Identification of 2-[2-nitro-4-[trifluoromethyl]benzoyl]-cyclohexane-1,3-dione metabolites in urine of patients suffering from tyrosinemia type I with the use of1H and 19F NMR spectroscopy

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Szczecinski P., Lamparska D., Gryff-Keller A., Gradowska W.
Acta Biochimica Polonica
|
2008
|
tom 55
|
nr 4
749-752
Organic extracts of six urine samples from children treated with nitisinone, a medicine against tyrosinemia type I, were investigated by 1H and 19F NMR spectroscopy. The presence of unchanged 2-[2-nitro-4-(trifluoromethyl)benzoyl]cyclohexane-1,3-dione (NTBC), 6-hydroxy-2-[2-nitro-4-(trifluoromethyl)benzoyl]cyclohexane-1,3-dione (NTBC-OH) and 2-nitro-4-trifluoromethylbenzoic acid (NTFA) as well as a few other unidentified compounds containing CF3 group was documented.
3

Tyrosine and its catabolites: from disease to cancer

100%
Tanguay R. M., Jorquera R., Poudrier J., St-Louis M.
Acta Biochimica Polonica
|
1996
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tom 43
|
nr 1
Hereditary tyrosinemia type I (HT I, McKusick 276700) is a metabolic disease with a pattern of autosomal recessive inheritance. The disease is caused by a deficiency of the enzyme involved in the last step in the degradation of the amino acid tyrosine, fumarylacetoacetate hydrolase (FAH). The result of this block is the accumulation of catabolites some of which have been proposed to be highly toxic due to their alkylating potential. In humans, hereditary tyrosinemia is often associated with the development of hepatocellular carcinoma in young patients. The reasons for the high incidence of hepatocellular carcinoma are unknown but it has been suggested that it may be caused by accumulated metabolites such as fumarylacetoacetate (FAA) and maieylace­toacetate (MAA). The various mutational defects in the FAH gene are reviewed. The use of two mouse models of this disease to study the molecular basis of the pathologies associated with HT I are discussed. Finally, some preliminary data on the mutagenic potential of FAA and MAA in a gene reversal assay are presented.
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