Search results for "Wild type"

showing 10 items of 181 documents

Substrate specificity overlap and interaction between Adrenoleukodystrophy protein (ALDP/ABCD1) and Adrenoleukodystrophy-related protein (ALDRP/ABCD2)

2011

X-linked adrenoleukodystrophy (X-ALD) is a neurodegenerative disorder caused by mutations in the ABCD1 gene, which encodes a peroxisomal member of the ATP-binding cassette (ABC) transporter subfamily D called ALDP. ALDP is supposed to function as a homodimer allowing the entry of CoA-esters of very-long chain fatty acids (VLCFA) into the peroxisome, the unique site of their β-oxidation. ALDP deficiency can be corrected by overexpression of ALDRP, its closest homolog. However, the exact nature of the substrates transported by ALDRP and its relationships with ALDP still remain unclear. To gain insight into the function of ALDRP, we used cell models allowing the induction in a dose-dependent m…

congenital hereditary and neonatal diseases and abnormalitiesendocrine system diseasesATP-binding cassette transportermembrane proteinsBiologyATP Binding Cassette Transporter Subfamily DBiochemistry03 medical and health sciences0302 clinical medicineabc transporterCell Line TumormedicineAnimals[SDV.NEU] Life Sciences [q-bio]/Neurons and Cognition [q-bio.NC]Molecular BiologyBeta oxidationfatty acid oxidation030304 developmental biologychemistry.chemical_classification0303 health sciencesadrenoleukodystrophyabc transporter;fatty acid;fatty acid oxidation;membrane proteins;peroxisomes;adrenoleukodystrophyFatty AcidsNeurosciencesWild typeFatty acidnutritional and metabolic diseasesperoxisomesCell BiologyPeroxisomemedicine.diseaseLipidsRatschemistryMembrane proteinBiochemistry[ SDV.NEU ] Life Sciences [q-bio]/Neurons and Cognition [q-bio.NC]Neurons and CognitionATP-Binding Cassette TransportersAdrenoleukodystrophy[SDV.NEU]Life Sciences [q-bio]/Neurons and Cognition [q-bio.NC]fatty acidOxidation-Reduction030217 neurology & neurosurgeryPolyunsaturated fatty acid
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Analysis of pteridines in Pyrrhocoris apterus (L.) (Heteroptera, Pyrrhocoridae) during development and in body-color mutants

1997

By using thin-layer chromatography (TLC) and high-performance liquid chromatography (HPLC), five different pteridines have been quantified in extracts from Pyrrhocoris apterus: neopterin, isoxanthopterin, isoxantholumazine (violapterin), 7-methylxanthopterin, and erythropterin. Biopterin was also detected using HPLC. Pteridines have been analyzed separately in bodies and eyes of the wild type regarding developmental stage and sex. The pteridine content in both bodies and eyes increased from nymphs to 2-day-old adults. After this period, the concentration of pteridines in the eyes of adults remained approximately constant, while in the bodies isoxantholumazine, 7-methylxanthopterin, and isox…

food.ingredientbiologyPhysiologyPyrrhocoridaeMutantWild typeNeopterinGeneral MedicinePyrrhocorisbiology.organism_classificationBiochemistryWhite (mutation)chemistry.chemical_compoundfoodBiochemistrychemistryInsect ScienceYolkBotanymedicinePteridinemedicine.drug
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Cholesterol-Streptolysin O Interaction: An EM Study of Wild-Type and Mutant Streptolysin O

1998

We present transmission electron microscopical data from negatively stained specimens of cholesterol following interaction with the thiol-activated bacterial toxin streptolysin O (SLO) (wild-type and a number of cysteine substitution mutants), with and without chemical modification of the cysteine residues. Two experimental systems were used, one with an aqueous suspension of cholesterol microcrystals and the other with immobilized thin planar cholesterol crystals attached to a carbon film. In both systems the wild-type SLO and two cytolytically active mutants, Cys 530 --Ala (C530A) and Ser 101 --Cys (S101C), readily generated the characteristic SLO arc- and ring-like oligomers on the surfa…

genetic structuresMutantWild typeChemical modificationOligomereye diseaseschemistry.chemical_compoundMonomerchemistryBiochemistryStructural BiologyBiotinylationBiophysicsStreptolysinsense organsCysteineJournal of Structural Biology
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Role of the nNOS gene in ethanol-induced conditioned place preference in mice

2009

Nitric oxide (NO) produced by neuronal nitric oxide synthase (nNOS) has a role in synaptic plasticity, and evidence suggests its role in a range of effects produced by alcohol in the central nervous system. The aim of the current study was to investigate the role of the nNOS gene in the development of ethanol-induced conditioned place preference (CPP) in mice. The CPP paradigm is designed to investigate the reinforcing properties of drugs of abuse and the development of maladaptive behaviors, such as conditioned response to drug-associated stimuli, following repeated drug exposure. Adult male and female wild type (WT) and nNOS knockout (KO) mice on a mixed B6; 129S genetic background were t…

inorganic chemicalsMalemedicine.medical_specialtyHealth (social science)medicine.medical_treatmentCentral nervous systemNitric Oxide Synthase Type IMotor ActivityToxicologyBiochemistryArticleNitric oxideBehavioral Neurosciencechemistry.chemical_compoundMiceInternal medicineConditioning PsychologicalmedicineAvoidance LearningAnimalsSalineMice KnockoutEthanolEthanolWild typeGeneral MedicineConditioned place preferenceAssociative learningbody regionsEndocrinologymedicine.anatomical_structureNeurologychemistrynervous systemSynaptic plasticitycardiovascular systemFemalePsychologyNeuroscience
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Structural, Thermodynamic, and Kinetic Traits of Antiestrogen-Compounds Selectively Targeting the Y537S Mutant Estrogen Receptor α Transcriptional Ac…

2019

The most frequently diagnosed cancers in women are the estrogen receptor (ER)-positive breast cancer subtypes, which are characterized by estrogen dependency for their growth. The mainstay of clinical treatment for this tumor relies on the modulation of ER action or on the suppression of estrogen biosynthesis via the administration of Selective ERα Modulators/Down-regulators (SERMs/SERDs) or aromatase inhibitors, respectively. Nevertheless, de novo and acquired resistance to these therapies frequently occurs and represents a major clinical concern for patient survival. Recently, somatic mutations affecting the hormone-binding domain of ERα (i. e. Y537S, Y537N, D538G) have been associated w…

medicine.drug_classSomatic cellIn silicoEstrogen receptor-02 engineering and technology010402 general chemistry01 natural scienceslcsh:ChemistryBreast cancerbreast cancermedicineAromataseresistant breast cancersOriginal ResearchbiologyChemistryWild typeY537SGeneral Chemistry021001 nanoscience & nanotechnologyAntiestrogenmedicine.diseaseSERMmolecular dynamics0104 chemical sciencesChemistrylcsh:QD1-999EstrogenSERDbiology.proteinCancer research0210 nano-technologyestrogen receptorFrontiers in chemistry
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Non-wild-type cryptococcosis in a child with multivisceral organ transplant who owned bird pets.

2020

Cryptococcosis is the third most common invasive fungal infection in solid-organ transplant recipients, although it is rarely reported in transplanted children. It typically appears as a late-onset infection with central nervous system or pulmonary involvement. We present a case of cryptococcosis caused by a non-wild strain in a 10-year-old child who owned two pet birds, and review the existent literature.

medicine.medical_specialtyAntifungal AgentsCentral nervous systemDrug resistance030230 surgeryOrgan transplantationBirds03 medical and health sciences0302 clinical medicinemedicineAnimalsHumansChildCryptococcus neoformansTransplantationNon wild typebiologybusiness.industryCryptococcosisOrgan TransplantationPetsbiology.organism_classificationmedicine.diseaseInfectious Diseasesmedicine.anatomical_structureImmunologyCryptococcosisCryptococcus neoformans030211 gastroenterology & hepatologySolid organ transplantationbusinessTransplant infectious disease : an official journal of the Transplantation SocietyREFERENCES
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Expression of Wild-Type and Variant Estrogen Receptor Alpha in Liver Carcinogenesis and Tumor Progression.

2011

Although estrogen receptors (ERs) are expressed in human hepatocellular carcinoma (HCC), several clinical trials have failed to demonstrate the efficacy of antiestrogen treatment in HCC patients. Recently, the identification of several ER splicing variants has enlightened the complex nature of estrogen signaling in peripheral tissues; this may help understanding estrogen role in either nontumoral or malignant nonclassical target organs, including liver. In this work we have investigated mRNA expression of wild-type and splice variants of ERα in nontumoral, cirrhotic, and malignant human liver, as well as in HCC cell lines, using an exon-specific reverse transcription polymerase chain reacti…

medicine.medical_specialtyCarcinoma Hepatocellularmedicine.drug_classEstrogen receptorBiologyBiochemistryAromataseCell Line TumorInternal medicineGene OrderGeneticsmedicineHumansRNA MessengerneoplasmsMolecular BiologyLiver NeoplasmsEstrogen Receptor alphaWild typeExonsHep G2 Cellsmedicine.diseaseAntiestrogenGene Expression Regulation NeoplasticReverse transcription polymerase chain reactionAlternative SplicingCell Transformation NeoplasticEndocrinologyLiverEstrogenTumor progressionHepatocellular carcinomaCancer researchMolecular MedicineEstrogen receptor alphaLiver carcinogenesis Estrogen receptors tumor progressionBiotechnology
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Penicillin induced epileptiform activity and EEG spectrum analysis of BDNF heterozygous mice: an in vivo electrophysiological study.

2011

Brain-derived neurotrophic factor (BDNF) heterozygous mice (BDNF (+/-)) kindle slowly and have a higher seizure threshold. However, BDNF (+/-) mice exhibit reduced cortical inhibition and disrupted balance of excitation/inhibition synaptic transmission. We investigated penicillin-induced focal cortical epileptiform activity and electroencephalogram (EEG) spectral power of BDNF (+/-) mice, by using electrocorticogram (ECoG) recordings. BDNF (+/-) mice (n=10) and wild type littermates (n=9) were anesthetized with i.p. urethane (1.750g/kg). The recordings of ECoG were carried out by using a data acquisition system and 100IU penicillin was administered intracortically to induce epileptiform act…

medicine.medical_specialtyHeterozygoteMicroinjectionsMice TransgenicNeocortexPenicillinsElectroencephalographyNeurotransmissionMiceNeurotrophic factorsInternal medicinemedicineAnimalsCerebral CortexMice KnockoutEpilepsymedicine.diagnostic_testSeizure thresholdChemistryGeneral NeuroscienceBrain-Derived Neurotrophic FactorWild typeElectroencephalographyCortex (botany)Electrophysiological PhenomenaElectrophysiologyEndocrinologymedicine.anatomical_structurenervous systemCerebral cortexNeuroscienceBrain research bulletin
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Derepressing muscleblind expression by miRNA sponges ameliorates myotonic dystrophy-like phenotypes in Drosophila

2016

AbstractMyotonic Dystrophy type 1 (DM1) originates from alleles of the DMPK gene with hundreds of extra CTG repeats in the 3′ untranslated region (3′ UTR). CUG repeat RNAs accumulate in foci that sequester Muscleblind-like (MBNL) proteins away from their functional target transcripts. Endogenous upregulation of MBNL proteins is, thus, a potential therapeutic approach to DM1. Here we identify two miRNAs, dme-miR-277 and dme-miR-304, that differentially regulate muscleblind RNA isoforms in miRNA sensor constructs. We also show that their sequestration by sponge constructs derepresses endogenous muscleblind not only in a wild type background but also in a DM1 Drosophila model expressing non-co…

musculoskeletal diseases0301 basic medicineUntranslated regioncongenital hereditary and neonatal diseases and abnormalitiesMotor ActivityBiologyMyotonic dystrophyArticle03 medical and health sciences0302 clinical medicineRNA IsoformsmicroRNAmedicineAnimalsDrosophila ProteinsMyotonic DystrophyRegulation of gene expressionGeneticsMultidisciplinaryWild typeNuclear Proteinsmedicine.diseaseMicroRNAsDrosophila melanogasterPhenotype030104 developmental biologyGene Expression RegulationFlight AnimalTrinucleotide Repeat ExpansionTrinucleotide repeat expansion030217 neurology & neurosurgeryDrosophila ProteinScientific Reports
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Association of different tumor necrosis factor α promoter allele frequencies with ankylosing spondylitis in HLA-B27 positive individuals

1998

OBJECTIVE To investigate the potential association of tumor necrosis factor alpha (TNFalpha) promoter alleles with ankylosing spondylitis. METHODS DNA from 141 HLA-B27 positive Caucasian patients with ankylosing spondylitis and 46 B27-positive and 99 B27-negative healthy Caucasian controls was investigated by polymerase chain reaction amplification of the TNFalpha promoter region and subsequent dot-blot analysis with allele-specific oligonucleotides. RESULTS There was a significant decrease in the promoter alleles TNF-238.2 and TNF-308.2 in the ankylosing spondylitis group (266 wild-type alleles, 16 variant alleles) compared with the B27-positive (75 wildtype promoter alleles, 17 variant al…

musculoskeletal diseasesAnkylosing spondylitisImmunologyHaplotypeWild typePromoterBiologymedicine.diseaseGenetic determinismRheumatologyImmunologymedicineImmunology and AllergyPharmacology (medical)Tumor necrosis factor alphaAlleleAllele frequencyArthritis & Rheumatism
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