2-{[2-nitro-4-(trifluoromethyl)phenyl]carbonyl}cyclohexane-1,3-dione
| Title | Journal |
|---|---|
| Single dose NTBC-treatment of hereditary tyrosinemia type I. | Journal of inherited metabolic disease 20120901 |
| Effect of nitisinone (NTBC) treatment on the clinical course of hepatorenal tyrosinemia in Québec. | Molecular genetics and metabolism 20120901 |
| Focal ligamentum flavum hypertrophy with ochronotic deposits: an unusual cause for neurogenic claudication in alkaptonuria. | Asian spine journal 20120601 |
| Simple and fast quantification of nitisone (NTBC) using liquid chromatography-tandem mass spectrometry method in plasma of tyrosinemia type 1 patients. | Journal of chromatographic science 20120501 |
| Diurnal variation of phenylalanine concentrations in tyrosinaemia type 1: should we be concerned? | Journal of human nutrition and dietetics : the official journal of the British Dietetic Association 20120401 |
| Neurocognitive outcome in patients with hypertyrosinemia type I after long-term treatment with NTBC. | Journal of inherited metabolic disease 20120301 |
| [Clinical follow up of Chilean patients with tyrosinemia type 1 treated with 2-(2-nitro-4-trifluoromethylbenzoyl)-1,3-ciclohexanedione (NTBC)]. | Revista medica de Chile 20120201 |
| LC-MS/MS method for simultaneous determination on a dried blood spot of multiple analytes relevant for treatment monitoring in patients with tyrosinemia type I. | Analytical chemistry 20120117 |
| A quantitative assessment of alkaptonuria: testing the reliability of two disease severity scoring systems. | Journal of inherited metabolic disease 20111201 |
| Natural history of alkaptonuria revisited: analyses based on scoring systems. | Journal of inherited metabolic disease 20111201 |
| Tyrosinemia type 1 in Spain: mutational analysis, treatment and long-term outcome. | Pediatrics international : official journal of the Japan Pediatric Society 20111201 |
| Alkaptonuria: treasure your exceptions. | Journal of inherited metabolic disease 20111201 |
| Medical management of chronic liver diseases in children (part I): focus on curable or potentially curable diseases. | Paediatric drugs 20111201 |
| Grown up mice from gene-corrected iPS cells. | Circulation research 20111111 |
| Informed reasoning: repositioning of nitisinone to treat oculocutaneous albinism. | The Journal of clinical investigation 20111003 |
| Nitisinone improves eye and skin pigmentation defects in a mouse model of oculocutaneous albinism. | The Journal of clinical investigation 20111003 |
| Neuropsychological outcome of NTBC-treated patients with tyrosinaemia type 1. | Developmental medicine and child neurology 20111001 |
| Long-term outcome of living donor liver transplantation in a Thai boy with hereditary tyrosinemia type I: a case report. | Journal of the Medical Association of Thailand = Chotmaihet thangphaet 20111001 |
| A 3-year randomized therapeutic trial of nitisinone in alkaptonuria. | Molecular genetics and metabolism 20110801 |
| Hereditary tyrosinemia type 1 from a single center in Egypt: clinical study of 22 cases. | World journal of pediatrics : WJP 20110801 |
| Generation of healthy mice from gene-corrected disease-specific induced pluripotent stem cells. | PLoS biology 20110701 |
| Comparison of plasma and dry blood spots as samples for the determination of nitisinone (NTBC) by high-performance liquid chromatography-tandem mass spectrometry. Study of the stability of the samples at different temperatures. | Journal of chromatography. B, Analytical technologies in the biomedical and life sciences 20110401 |
| Increase of CSF tyrosine and impaired serotonin turnover in tyrosinemia type I. | Molecular genetics and metabolism 20110201 |
| Monitoring tyrosinaemia type I: Blood spot test for nitisinone (NTBC). | Clinica chimica acta; international journal of clinical chemistry 20110114 |
| Alkaptonuria. | Indian journal of dermatology 20110101 |
| A novel ex vivo organotypic culture model of alkaptonuria-ochronosis. | Clinical and experimental rheumatology 20110101 |
| Hepatic differentiation of murine disease-specific induced pluripotent stem cells allows disease modelling in vitro. | Stem cells international 20110101 |
| Alkaptonuria. | Dermatology online journal 20101115 |
| A late and difficult diagnosis of ochronosis. | Journal of medicine and life 20101115 |
| Product analysis and inhibition studies of a causative Asn to Ser variant of 4-hydroxyphenylpyruvate dioxygenase suggest a simple route to the treatment of Hawkinsinuria. | Biochemistry 20100824 |
| Tyrosinemia type 1: metastatic hepatoblastoma with a favorable outcome. | Pediatrics 20100701 |
| Clinical practice. NTBC therapy for tyrosinemia type 1: how much is enough? | European journal of pediatrics 20100601 |
| Significant increase of succinylacetone within the first 12 h of life in hereditary tyrosinemia type 1. | European journal of pediatrics 20100501 |
| High volume naked DNA tail-vein injection restores liver function in Fah-knock out mice. | Journal of gastroenterology and hepatology 20100501 |
| Determination of NTBC in serum samples from patients with hereditary tyrosinemia type I by capillary electrophoresis. | Talanta 20100315 |
| Identification of NTBC metabolites in urine from patients with hereditary tyrosinemia type 1 using two different mass spectrometric platforms: triple stage quadrupole and LTQ-Orbitrap. | Rapid communications in mass spectrometry : RCM 20100301 |
| A metabolic cause of spinal deformity. | Metabolism: clinical and experimental 20100101 |
| [Clinical, biochemical and molecular characteristics in 11 Czech children with tyrosinemia type I]. | Casopis lekaru ceskych 20100101 |
| Persistent coagulopathy during Escherichia coli sepsis in a previously healthy infant revealed undiagnosed tyrosinaemia type 1. | BMJ case reports 20100101 |
| Liquid chromatography tandem mass spectrometry method for the quantitation of NTBC (2-(nitro-4-trifluoromethylbenzoyl)1,3-cyclohexanedione) in plasma of tyrosinemia type 1 patients. | Journal of chromatography. B, Analytical technologies in the biomedical and life sciences 20090515 |
| Inhibition of 4-hydroxyphenylpyruvate dioxygenase by 2-[2-nitro-4-(trifluoromethyl)benzoyl]-1,3-cyclohexanedione. | Acta biochimica Polonica 20090101 |
| No difference in between-country variability in use of newly approved orphan and non- orphan medicinal products--a pilot study. | Orphanet journal of rare diseases 20090101 |
| A novel mutation causing mild, atypical fumarylacetoacetase deficiency (Tyrosinemia type I): a case report. | Orphanet journal of rare diseases 20090101 |
| Ochronosis of hip joint; a case report. | Cases journal 20090101 |
| Ochronosis as an unusual cause of valvular defect: a case report. | Journal of medical case reports 20090101 |
| Severe neurological crisis in a patient with hereditary tyrosinaemia type I after interruption of NTBC treatment. | Journal of inherited metabolic disease 20081201 |
| Activation of nuclear factor E2-related factor 2 in hereditary tyrosinemia type 1 and its role in survival and tumor development. | Hepatology (Baltimore, Md.) 20080801 |
| Loss of p21 permits carcinogenesis from chronically damaged liver and kidney epithelial cells despite unchecked apoptosis. | Cancer cell 20080708 |
| Renal tubular function in children with tyrosinaemia type I treated with nitisinone. | Journal of inherited metabolic disease 20080601 |
| Experience of nitisinone for the pharmacological treatment of hereditary tyrosinaemia type 1. | Expert opinion on pharmacotherapy 20080501 |
| Rescue from neonatal death in the murine model of hereditary tyrosinemia by glutathione monoethylester and vitamin C treatment. | Molecular genetics and metabolism 20080301 |
| Hepatic stress in hereditary tyrosinemia type 1 (HT1) activates the AKT survival pathway in the fah-/- knockout mice model. | Journal of hepatology 20080201 |
| NTBC treatment in tyrosinaemia type I: long-term outcome in French patients. | Journal of inherited metabolic disease 20080201 |
| Alkaptonuria diagnosed in a 4-month-old baby girl: a case report. | Cases journal 20080101 |
| 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 of 1H and 19F NMR spectroscopy. | Acta biochimica Polonica 20080101 |
| Messenger RNA as a source of transposase for sleeping beauty transposon-mediated correction of hereditary tyrosinemia type I. | Molecular therapy : the journal of the American Society of Gene Therapy 20070701 |
| Nitisinone: new drug. Type 1 tyrosinemia: an effective drug. | Prescrire international 20070401 |
| In silico prediction of pregnane X receptor activators by machine learning approaches. | Molecular pharmacology 20070101 |
| Tyrosinemia type I treated by NTBC: how does AFP predict liver cancer? | Molecular genetics and metabolism 20061201 |
| [New drugs; nitisinone]. | Nederlands tijdschrift voor geneeskunde 20061118 |
| Harnessing a high cargo-capacity transposon for genetic applications in vertebrates. | PLoS genetics 20061101 |
| Tyrosinemia produced by 2-(2-nitro-4-trifluoromethylbenzoyl)-cyclohexane-1,3-dione (NTBC) in experimental animals and its relationship to corneal injury. | Toxicology and applied pharmacology 20060815 |
| Lectin-reactive alpha-fetoprotein in patients with tyrosinemia type I and hepatocellular carcinoma. | Journal of pediatric gastroenterology and nutrition 20060701 |
| Evaluation of dichloroacetate treatment in a murine model of hereditary tyrosinemia type 1. | Biochemical pharmacology 20060528 |
| The genetic tyrosinemias. | American journal of medical genetics. Part C, Seminars in medical genetics 20060515 |
| Quantitative determination of succinylacetone in dried blood spots for newborn screening of tyrosinemia type I. | Molecular genetics and metabolism 20060501 |
| Response of metastatic recurrent neuroblastoma to nitisinone: a modulator of tyrosine metabolism. | Pediatric blood & cancer 20060401 |
| Involvement of endoplasmic reticulum stress in hereditary tyrosinemia type I. | The Journal of biological chemistry 20060303 |
| Kidneys of mice with hereditary tyrosinemia type I are extremely sensitive to cytotoxicity. | Pediatric research 20060301 |
| Gateways to clinical trials. | Methods and findings in experimental and clinical pharmacology 20060301 |
| Cardiomyopathy in tyrosinaemia type I is common but usually benign. | Journal of inherited metabolic disease 20060201 |
| Current strategies for the treatment of hereditary tyrosinemia type I. | Paediatric drugs 20060101 |
| Nitisinone in the treatment of hereditary tyrosinaemia type 1. | Drugs 20060101 |
| Drug discovery from medicinal plants. | Life sciences 20051222 |
| Spectroscopic and computational studies of NTBC bound to the non-heme iron enzyme (4-hydroxyphenyl)pyruvate dioxygenase: active site contributions to drug inhibition. | Biochemical and biophysical research communications 20051209 |
| Transcriptome analysis of Paracoccidioides brasiliensis cells undergoing mycelium-to-yeast transition. | Eukaryotic cell 20051201 |
| Use of nitisinone in patients with alkaptonuria. | Metabolism: clinical and experimental 20050601 |
| Tandem mass spectrometric assay of succinylacetone in urine for the diagnosis of hepatorenal tyrosinemia. | Analytical biochemistry 20050415 |
| New developments in ochronosis: review of the literature. | Rheumatology international 20050301 |
| Successful treatment of severe cardiomyopathy with NTBC in a child with tyrosinaemia type I. | Journal of inherited metabolic disease 20050101 |
| [Lectin-reactive alpha-fetoprotein in tyrosinaemia type I]. | Klinische Padiatrie 20050101 |
| Hepatomegaly: commentary. | Clinical pediatrics 20050101 |
| Liquid chromatography-tandem mass spectrometry method for the simultaneous determination of delta-ALA, tyrosine and creatinine in biological fluids. | Clinica chimica acta; international journal of clinical chemistry 20041201 |
| Alkaptonuric ochronosis with aortic valve and joint replacements and femoral fracture: a case report and literature review. | Clinical medicine & research 20041101 |
| D-serine-induced nephrotoxicity: possible interaction with tyrosine metabolism. | Toxicology 20040901 |
| Renal proximal tubular cells acquire resistance to cell death stimuli in mice with hereditary tyrosinemia type 1. | Kidney international 20040901 |
| Experience with NTBC therapy in hereditary tyrosinaemia type I: an alternative to liver transplantation. | Annals of tropical paediatrics 20040901 |
| Structure of the ferrous form of (4-hydroxyphenyl)pyruvate dioxygenase from Streptomyces avermitilis in complex with the therapeutic herbicide, NTBC. | Biochemistry 20040601 |
| Chronic liver disease in murine hereditary tyrosinemia type 1 induces resistance to cell death. | Hepatology (Baltimore, Md.) 20040201 |
| Extensive changes in liver gene expression induced by hereditary tyrosinemia type I are not normalized by treatment with 2-(2-nitro-4-trifluoromethylbenzoyl)-1,3-cyclohexanedione (NTBC). | Journal of hepatology 20031201 |
| 4-Hydroxyphenylpyruvate dioxygenase as a drug discovery target. | Drug news & perspectives 20031001 |
| Interaction of (4-hydroxyphenyl)pyruvate dioxygenase with the specific inhibitor 2-[2-nitro-4-(trifluoromethyl)benzoyl]-1,3-cyclohexanedione. | Biochemistry 20030902 |
| Pharmacological rescue of the 14CoS/14CoS mouse: hepatocyte apoptosis is likely caused by endogenous oxidative stress. | Free radical biology & medicine 20030815 |
| Reversibility of cirrhotic regenerative liver nodules upon NTBC treatment in a child with tyrosinaemia type I. | Acta paediatrica (Oslo, Norway : 1992) 20030501 |
| The role of mode of action studies in extrapolating to human risks in toxicology. | Toxicology letters 20030411 |
| Alkaptonuria. | The New England journal of medicine 20030403 |
| Ophthalmic follow-up of patients with tyrosinaemia type I on NTBC. | Journal of inherited metabolic disease 20030101 |
| Natural history of alkaptonuria. | The New England journal of medicine 20021226 |
| Goldenhar syndrome and hereditary tyrosinemia type 1. | Saudi medical journal 20021201 |
| Corneal opacities associated with NTBC treatment. | American journal of ophthalmology 20020801 |
| Tyrosinemia I, a model for human diseases mediated by 2-oxoacid-utilizing dioxygenases: hepatotoxin suppression by NTBC does not normalize hepatic collagen metabolism. | Journal of pediatric gastroenterology and nutrition 20020701 |
| From the Food and Drug Administration. | JAMA 20020306 |
| Long-term therapy with NTBC and tyrosine-restricted diet in a murine model of hereditary tyrosinemia type I. | Molecular genetics and metabolism 20020101 |
| Drug to treat rare pediatric liver disease. | FDA consumer 20020101 |
| Nitisinone. Ntbc, Orfadin. | Drugs in R&D 20020101 |
| Pharmacokinetics and pharmacodynamics of NTBC (2-(2-nitro-4-fluoromethylbenzoyl)-1,3-cyclohexanedione) and mesotrione, inhibitors of 4-hydroxyphenyl pyruvate dioxygenase (HPPD) following a single dose to healthy male volunteers. | British journal of clinical pharmacology 20010801 |
| [Evolution of a case of tyrosinemia type I treated with NTBC]. | Anales espanoles de pediatria 20010301 |
| SAR studies of 2-o-substituted-benzoyl- and 2-alkanoyl-cyclohexane-1,3-diones as inhibitors of 4-hydroxyphenylpyruvate dioxygenase. | Bioorganic & medicinal chemistry letters 20000501 |