The β-triketone, nitisinone, kills insecticide-resistant mosquitoes through cuticular uptake [PDF]
Parasites & VectorsBackground Insecticide resistance in disease-transmitting arthropods of agricultural, veterinary, and public health significance poses a significant threat to vector control programs worldwide.
Zachary Thomas Stavrou-Dowd +6 more
doaj +4 more sources
Type 1 tyrosinemia in Finland: a nationwide study [PDF]
Orphanet Journal of Rare Diseases, 2020 Background Introduction of nitisinone and newborn screening (NBS) have transformed the treatment of type 1 tyrosinemia, but the effects of these changes on the long-term outcomes remain obscure.
Linnea Äärelä +8 more
doaj +6 more sources
Inter‐laboratory analytical improvement of succinylacetone and nitisinone quantification from dried blood spot samples [PDF]
JIMD Reports, 2020 Background Nitisinone is used to treat hereditary tyrosinemia type 1 (HT‐1) by preventing accumulation of toxic metabolites, including succinylacetone (SA). Accurate quantification of SA during newborn screening is essential, as is quantification of both
Hilde Laeremans +9 more
doaj +2 more sources
Molecular Genetics and Metabolism Reports, 2022 Background: Although changes in the tyrosine pathway during nitisinone therapy are known, a complete characterization of the induced tyrosinaemia is lacking to improve disease management.
L.R. Ranganath +13 more
doaj +3 more sources
JIMD Reports, 2022 Background Outcomes from studies employing nitisinone 10 mg and 2 mg in alkaptonuria were compared. Patients and methods Sixty‐nine patients in each of the nitisinone (10 mg daily) and controls of suitability of nitisinone in alkaptonuria 2 (SONIA 2), as
Lakshminarayan R. Ranganath +10 more
doaj +2 more sources
The Discovery of the Mode of Action of Nitisinone
Metabolites, 2022 This review briefly discusses the discovery of the mode of action of the triketone herbicide, 2-(2-nitro-4-trifluormethylbenzoyl)-1,3-cyclohexanedione and its use as a drug Nitisinone for the treatment of inborn errors of tyrosine metabolism.
Edward A. Lock
doaj +3 more sources
Preventive use of nitisinone in alkaptonuria [PDF]
Orphanet Journal of Rare Diseases, 2021 Alkaptonuria (AKU, OMIM 203500) is a rare congenital disorder caused by a deficiency of the enzyme homogentisate-1,2,-dioxygenase. The long-term consequences of AKU are joint problems, cardiac valve abnormalities and renal problems. Landmark intervention
Bruce H. R. Wolffenbuttel +2 more
doaj +2 more sources
Comprehensive Biotransformation Analysis of Phenylalanine-Tyrosine Metabolism Reveals Alternative Routes of Metabolite Clearance in Nitisinone-Treated Alkaptonuria [PDF]
Metabolites, 2022 Metabolomic analyses in alkaptonuria (AKU) have recently revealed alternative pathways in phenylalanine-tyrosine (phe-tyr) metabolism from biotransformation of homogentisic acid (HGA), the active molecule in this disease.
Brendan P. Norman +12 more
doaj +4 more sources
Evaluating the impact of nitisinone at mosquito-lethal doses on Lutzomyia longipalpis. [PDF]
PLoS Neglected Tropical DiseasesNitisinone, a systemic inhibitor of tyrosine catabolism, has recently emerged as a promising endectocide with demonstrated lethality against mosquitoes and tsetse flies.
Laure Augendre +5 more
doaj +2 more sources
An Unusual Presentation of Tyrosinemia Type 1 in a Pediatric Patient: Case Report and Comprehensive Review [PDF]
Clinical Case ReportsTyrosinemia type 1 often manifests with liver, renal, or peripheral neuropathy disorders. Before therapies like nitisinone, management was limited to dietary modifications and liver transplantation.
Mahsa Rouhafshari +4 more
doaj +2 more sources