Search results for "Pterins"

showing 5 items of 5 documents

Cyclic AMP-mediated upregulation of the expression of neuronal NO synthase in human A673 neuroepithelioma cells results in a decrease in the level of…

2004

The expression level of neuronal nitric oxide synthase (nNOS) can vary depending on the (patho)physiological conditions. Here we document a marked induction of nNOS mRNA, protein, and total NO production in response to dibutyryl cyclic AMP (db-cAMP) in human A673 neuroepithelial cells. However, the upregulation of nNOS was associated with a decreased level of production of bioactive NO and by an increase in the level of generation of reactive oxygen species (ROS). ROS production could be prevented by the NOS inhibitor L-NAME, suggesting nNOS itself is involved in ROS generation. Sepiapterin supplementation of db-cAMP-treated A673 cells could restore full bioactive NO production, most likely…

CAMP-Responsive Element ModulatorNitric Oxide Synthase Type IBiologyCREBNitric OxideBiochemistryAdenylyl cyclaseCyclic AMP Response Element Modulatorchemistry.chemical_compoundMiceNeuroblastomaCoactivatorComplement C3b Inactivator ProteinsCyclic AMPAnimalsHumansNeuroectodermal Tumors Primitive PeripheralCREB-binding proteinEnzyme InhibitorsProtein kinase AeducationCyclic AMP Response Element-Binding ProteinGTP CyclohydrolaseCAMP response element bindingHomeodomain ProteinsNeuronseducation.field_of_studyForskolinPhosphoric Diester HydrolasesIntracellular Signaling Peptides and ProteinsBlood ProteinsLIM Domain ProteinsMolecular biologyCyclic AMP-Dependent Protein KinasesPterinsUp-RegulationDNA-Binding ProteinsRepressor ProteinsAntisense Elements (Genetics)NG-Nitroarginine Methyl EsterchemistryBucladesineGene Expression RegulationComplement Factor Hbiology.proteinNitric Oxide SynthaseReactive Oxygen SpeciesSignal TransductionBiochemistry
researchProduct

Efficacy and safety of cyclic pyranopterin monophosphate substitution in severe molybdenum cofactor deficiency type A: a prospective cohort study.

2015

Summary Background Molybdenum cofactor deficiency (MoCD) is characterised by early, rapidly progressive postnatal encephalopathy and intractable seizures, leading to severe disability and early death. Previous treatment attempts have been unsuccessful. After a pioneering single treatment we now report the outcome of the complete first cohort of patients receiving substitution treatment with cyclic pyranopterin monophosphate (cPMP), a biosynthetic precursor of the cofactor. Methods In this observational prospective cohort study, newborn babies with clinical and biochemical evidence of MoCD were admitted to a compassionate-use programme at the request of their treating physicians. Intravenous…

Compassionate Use TrialsMalePediatricsmedicine.medical_specialtyGENOMIC STRUCTUREFEATURESEncephalopathyMolybdopterin synthaseCyclic pyranopterin monophosphateDrug Administration ScheduleCohort Studieschemistry.chemical_compoundOrganophosphorus CompoundsmedicineURINEHumansBIOSYNTHESISProspective cohort studyAdverse effectMolybdenum cofactor deficiencyPRECURSORMetal Metabolism Inborn ErrorsMetal metabolismbusiness.industryMUTATIONSInfant NewbornGENES MOCS1CPMPMOLYBDOPTERIN SYNTHASEGeneral Medicinemedicine.diseasePterinsTreatment OutcomechemistryFemalebusinessCohort studyLancet (London, England)
researchProduct

Identification of 5,6,7,8-tetrahydropterin and 5,6,7,8-tetrahydrobiopterin in Drosophila melanogaster.

1988

Summary Using reversed-phase high-performance liquid chromatography with electrochemical detection we have demonstrated the occurrence of 5,6,7,8-tetrahydropterin and 5,6,7,8-tetrahydrobiopterin in Drosophila melanogaster . The former is the first time that has been detected in vivo . The identification has been based on the retention times, hydrodinamic voltagrams and the differential concentration in three strains of Drosophila melanogaster . Compared to the wild type, the Punch 2 mutant has diminished levels of both pteridines, whereas Henna-recessive 3 lacks completely tetrahydropterin and has increased levels of tetrahydrobiopterin, as expected according to their biochemical lesions.

GeneticsbiologyMutantBiophysicsWild typeCell BiologyElectrochemical detectionTetrahydrobiopterinbiology.organism_classificationKidneyBiochemistryHigh-performance liquid chromatographyBiopterinPterinsRatsDrosophila melanogasterBiochemistryIn vivomedicineAnimalsDrosophila melanogasterMolecular BiologyChromatography High Pressure Liquidmedicine.drugBiochemical and biophysical research communications
researchProduct

Epistatic interactions between pterin and carotenoid genes modulate intra-morph color variation in a lizard.

2021

Color polymorphisms have become a major topic in evolutionary biology and substantial efforts have been devoted to the understanding of the mechanisms responsible for originating such colorful systems. Within-morph continuous variation, on the other hand, has been neglected in most of the studies. Here, we combine spectrophotometric/visual modeling and genetic data to study the mechanisms promoting continuous variation within categorical color morphs of Podarcis muralis. Our results suggest that intra-morph variability in the pterin-based orange morph is greater compared to white and yellow morphs. We also show that continuous variation within the orange morph is partially discriminable by …

animal structuresgenetic structuresColorLocus (genetics)Biologychemistry.chemical_compoundbiology.animalAnimalsPterinAllelereproductive and urinary physiologyPolymorphism GeneticLizardPigmentationfungiLizardsbiology.organism_classificationBiological EvolutionCarotenoidsPterinsWhite (mutation)Podarcis muralisVariation (linguistics)chemistryEvolutionary biologyEpistasisAnimal Science and Zoologypsychological phenomena and processesIntegrative zoologyREFERENCES
researchProduct

Regulatory changes in pterin and carotenoid genes underlie balanced color polymorphisms in the wall lizard

2019

Significance Reptiles show an amazing color diversity based on variation in melanins, carotenoids, and pterins. This study reveals genes controlling differences between three color morphs (white, orange, and yellow) in the common wall lizard. Orange pigmentation, due to high levels of orange/red pterins in skin, is caused by genetic changes in the sepiapterin reductase gene. Yellow skin, showing high levels of yellow carotenoids, is controlled by the beta-carotene oxygenase 2 locus. Thus, the color polymorphism in the common wall lizard is associated with changes in two small regions of the genome containing genes with crucial roles in pterin and carotenoid metabolism. These genes are likel…

balanced polymorphismBalanced polymorphismgenetic structuresEvolutionIntrogressionintrogressionColorpterin pigmentationSkin PigmentationDioxygenasesEvolutionsbiologiGeneticAnimalscarotenoid pigmentationPolymorphismPterin pigmentationEvolutionary BiologyPolymorphism GeneticBalanced polymorphism; Carotenoid pigmentation; Introgression; Podarcis muralis; Pterin pigmentation; Alcohol Oxidoreductases; Animals; Carotenoids; Color; Dioxygenases; Lizards; Pigmentation; Polymorphism Genetic; Pterins; Skin PigmentationPigmentationLizardsBiological SciencesCarotenoidsPterinsAlcohol OxidoreductasesPNAS PlusCarotenoid pigmentationPodarcis muralissense organs
researchProduct