Search results for "Research"

showing 10 items of 25967 documents

C2orf69 mutations disrupt mitochondrial function and cause a multisystem human disorder with recurring autoinflammation

2021

BACKGROUND. Deciphering the function of the many genes previously classified as uncharacterized open reading frame (ORF) would complete our understanding of a cell’s function and its pathophysiology. METHODS. Whole-exome sequencing, yeast 2-hybrid and transcriptome analyses, and molecular characterization were performed in this study to uncover the function of the C2orf69 gene. RESULTS. We identified loss-of-function mutations in the uncharacterized C2orf69 gene in 8 individuals with brain abnormalities involving hypomyelination and microcephaly, liver dysfunction, and recurrent autoinflammation. C2orf69 contains an N-terminal signal peptide that is required and sufficient for mitochondrial…

0301 basic medicineMicrocephalyRespiratory chainBiologyMitochondrionCell LineMitochondrial ProteinsTranscriptomeMiceOpen Reading Frames03 medical and health sciencesAll institutes and research themes of the Radboud University Medical Center0302 clinical medicineLoss of Function MutationGlycogen branching enzymemedicineAnimalsHumansGeneMice KnockoutGeneticsMetabolic Disorders Radboud Institute for Molecular Life Sciences [Radboudumc 6]Glycogen Debranching Enzyme SystemGeneral Medicinemedicine.diseaseMitochondriaOpen reading frameRenal disorders Radboud Institute for Molecular Life Sciences [Radboudumc 11]030104 developmental biology030220 oncology & carcinogenesisMicrocephalybiology.proteinClinical MedicineSignal transductionGlycogenJournal of Clinical Investigation
researchProduct

Rhodococcus aetherivorans BCP1 as cell factory for the production of intracellular tellurium nanorods under aerobic conditions

2016

Tellurite (TeO3 2−) is recognized as a toxic oxyanion to living organisms. However, mainly anaerobic or facultative-anaerobic microorganisms are able to tolerate and convert TeO3 2− into the less toxic and available form of elemental Tellurium (Te0), producing Te-deposits or Te-nanostructures. The use of TeO3 2−-reducing bacteria can lead to the decontamination of polluted environments and the development of “green-synthesis” methods for the production of nanomaterials. In this study, the tolerance and the consumption of TeO3 2− have been investigated, along with the production and characterization of Te-nanorods by Rhodococcus aetherivorans BCP1 grown under aerobic conditions. Aerobically …

0301 basic medicineMicroorganism030106 microbiologyOxyanionBioengineeringSettore BIO/19 - Microbiologia GeneraleApplied Microbiology and Biotechnology03 medical and health scienceschemistry.chemical_compoundMinimum inhibitory concentrationBiogenic nanostructuresTelluriteRhodococcusFood scienceTellurium nanorodsSettore CHIM/02 - Chimica FisicaNanorods biosynthesisNanotubesbiologyStrain (chemistry)ResearchBiogenic nanostructureNanorods biosynthesiAerobiosiRhodococcus aetherivoranElemental telluriumTellurium nanorodbiology.organism_classificationAerobiosisNanotubeRhodococcus aetherivoranschemistryBiochemistryTelluriumAnaerobic exerciseRhodococcusBacteriaIntracellularRhodococcuBiotechnology
researchProduct

Studying nerve transfers: Searching for a consensus in nerve axons count

2021

Axonal count is the base for efficient nerve transfer; despite its capital importance, few studies have been published on human material, most research approaches being performed on experimental animal models of nerve injury. Thus, standard analysis methods are still lacking. Quantitative data obtained have to be reproducible and comparable with published data by other research groups. To share results with the scientific community, the standardization of quantitative analysis is a fundamental step. For this purpose, the experiences of the Italian, Austrian, German, Greek, and Iberian-Latin American groups have been compared with each other and with the existing literature to reach a consen…

0301 basic medicineMicrosurgeryNerve injurymedicine.medical_specialtyConsensusFuture studiesResearch groupsStandardizationSettore MED/19 - Chirurgia PlasticaCell Count03 medical and health sciences0302 clinical medicineAnimalsHumansMedicineAutograftsNerve TransferProtocol (science)Fiber countStaining and LabelingNerve transfersbusiness.industryHistological TechniquesExperimental Animal ModelsNerve injuryData scienceAxonsSurgeryEuropeLatin America030104 developmental biologyQuantitative analysis (finance)Fiber count; Microsurgery; Nerve injury; Nerve transfersNerve TransferSurgerymedicine.symptombusiness030217 neurology & neurosurgeryJournal of Plastic, Reconstructive & Aesthetic Surgery
researchProduct

MTA apical barrier: In vitro study of the use of ultrasonic vibration

2016

Background: The apexification is the first alternative treatment on a permanent tooth when, after a tooth trauma and in the presence of immature apex trauma, pulp necrosis occurs. Many studies have demonstrated the efficacy of mineral trioxide aggregate (MTA) as apical sealing material of choice in these cases, but has a degree of filtration as all other materials. The objective of this study was to analyze the seal ability of MTA on the duct walls in immature teeth unirradicular apexes, using indirect vibration. Material and Methods: The study was conducted on 45 teeth divided into 3 groups: Group A or control group in which no vibration for placing the MTA was used, Group B and C or group…

0301 basic medicineMineral trioxide aggregateMateriales dentalesDentistryOdontologíaUltrasonidos en medicinaOperative Dentistry and Endodontics03 medical and health sciences0302 clinical medicinestomatognathic systemUltrasonic vibrationIn vitro studyTecnología médicaGeneral DentistryPulp necrosisPermanent toothChemistrybusiness.industryResearch030206 dentistry:CIENCIAS MÉDICAS [UNESCO]Ciencias de la saludAlternative treatmentstomatognathic diseases030104 developmental biologyUNESCO::CIENCIAS MÉDICASApexificationbusinessJournal of Clinical and Experimental Dentistry
researchProduct

Vertical root fracture resistance of simulated immature permanent teeth filled with MTA using different vehicles

2017

Background The aim of the study is to evaluate the resistance vertical root fracture (VRF) of mineral trioxide aggregate (MTA) filled-immature permanent roots by using three different vehicles. Material and Methods Forty-extracted human single-rooted mandibular premolars were selected and the root length was standardized to the length of 9 mm. For simulation of immature tooth apices, peeso reamers were introduced into the root canals and the prepared roots were assigned into three experimental groups according the used vehicle (distilled water-DW, prophylene glycol-PG, chlorhexidine-CHX) and control group (n=10). To simulate a periodontal membrane, the apical 7 mm of all roots was covered w…

0301 basic medicineMineral trioxide aggregateMaterials scienceDentistryOdontologíaOperative Dentistry and Endodontics03 medical and health sciences0302 clinical medicineVertical root fractureFlexural strengthmedicineStatistical analysisGeneral DentistryPermanent teethUniversal testing machinebusiness.industryResearch030206 dentistryTooth Apexmedicine.disease:CIENCIAS MÉDICAS [UNESCO]Ciencias de la salud030104 developmental biologyUNESCO::CIENCIAS MÉDICASFracture (geology)business
researchProduct

The effects of silver nanoparticles on antimicrobial activity of ProRoot mineral trioxide aggregate (MTA) and calcium enriched mixture (CEM)

2016

Background: Although, mineral trioxide aggregate (MTA) and new experimental cement (CEM) are good root filling cements, but had no or low antimicrobial activities. The aim of this study was to evaluate the effects of addition of silver nanoparticles (SNP) to these two cements on antimicrobial effects against five most dental infection related microorganisms. Material and Methods: Two suspensions of 100 and 200 ppm of SNP were prepared and 180 μl of microbial suspension with 1.5 × 10 8 CFU/ml of each respected microorganisms were re-suspended in deionized water or each of SNP suspensions. After that, 60 μg of MTA and CEM were added to each tube. In one tube, the mixture of all above mentione…

0301 basic medicineMineral trioxide aggregateMicroorganismDentistryOdontologíaEnterococcus faecalisSilver nanoparticleAgar plate03 medical and health sciences0302 clinical medicineBiomaterials and Bioengineering in DentistryFood scienceGeneral DentistryCementbiologybusiness.industryChemistryResearch030206 dentistrybiology.organism_classificationAntimicrobial:CIENCIAS MÉDICAS [UNESCO]Ciencias de la salud030104 developmental biologyUNESCO::CIENCIAS MÉDICASAntibacterial activitybusiness
researchProduct

Effect of Smear Clear and Some Other Commonly Used Irrigants on dislodgement resistance of Mineral Trioxide Aggregate to Root Dentin

2016

Background This study aimed to assess the push-out bond strength of mineral trioxide aggregate (MTA) to root canal dentin after irrigation with Smear Clear in comparison with 2.5% sodium hypochlorite (NaOCl), 2% chlorhexidine (CHX) and saline as commonly used root canal irrigants. Material and Methods The coronal and mid-root areas of maxillary anterior teeth were horizontally sectioned into one-millimeter thick slices. The root canal lumen of dentinal slices was dilated using a diamond bur with 1.3 mm diameter. After the application of MTA, the samples were incubated in 100% humidity for 10 minutes and were then randomly divided into four groups (n=20) and immersed in Smear Clear, 2.5% NaO…

0301 basic medicineMineral trioxide aggregateRoot canalDentistryOperative Dentistry and Endodontics03 medical and health scienceschemistry.chemical_compound0302 clinical medicineDentinmedicineGeneral DentistryAnterior teethUniversal testing machineBond strengthbusiness.industryResearchChlorhexidine030206 dentistry:CIENCIAS MÉDICAS [UNESCO]030104 developmental biologymedicine.anatomical_structurechemistrySodium hypochloriteUNESCO::CIENCIAS MÉDICASbusinessmedicine.drugJournal of Clinical and Experimental Dentistry
researchProduct

Cellular Responses in Human Dental Pulp Stem Cells Treated with Three Endodontic Materials

2017

Human dental pulp stem cells (HDPSCs) are of special relevance in future regenerative dental therapies. Characterizing cytotoxicity and genotoxicity produced by endodontic materials is required to evaluate the potential for regeneration of injured tissues in future strategies combining regenerative and root canal therapies. This study explores the cytotoxicity and genotoxicity mediated by oxidative stress of three endodontic materials that are widely used on HDPSCs: a mineral trioxide aggregate (MTA-Angelus white), an epoxy resin sealant (AH-Plus cement), and an MTA-based cement sealer (MTA-Fillapex). Cell viability and cell death rate were assessed by flow cytometry. Oxidative stress was m…

0301 basic medicineMineral trioxide aggregatelcsh:Internal medicineArticle SubjectDNA damageDentistrymedicine.disease_cause03 medical and health sciences0302 clinical medicineDental pulp stem cellsmedicineViability assaylcsh:RC31-1245Molecular Biologybusiness.industryChemistryRegeneration (biology)030206 dentistryCell Biology030104 developmental biologyCell cultureCancer researchbusinessGenotoxicityOxidative stressResearch ArticleStem Cells International
researchProduct

Re-definition and supporting evidence toward Fanconi Anemia as a mitochondrial disease: Prospects for new design in clinical management

2021

Fanconi anemia (FA) has been investigated since early studies based on two definitions, namely defective DNA repair and proinflammatory condition. The former definition has built up the grounds for FA diagnosis as excess sensitivity of patients' cells to xenobiotics as diepoxybutane and mitomycin C, resulting in typical chromosomal abnormalities. Another line of studies has related FA phenotype to a prooxidant state, as detected by both in vitro and ex vivo studies. The discovery that the FA group G (FANCG) protein is found in mitochondria (Mukhopadhyay et al., 2006) has been followed by an extensive line of studies providing evidence for multiple links between other FA gene products and mi…

0301 basic medicineMitochondrial DNAMitochondrial DiseasesMitomycinMitochondrial diseaseClinical BiochemistryDiepoxybutaneReview ArticleMitochondrionBiologyBiochemistry03 medical and health scienceschemistry.chemical_compound0302 clinical medicineFanconi anemiaFANCGmedicineHumansClastogenCarnitinelcsh:QH301-705.5Coenzyme Q10lcsh:R5-920ProteinOrganic ChemistryMitochondrial nutrientProteinsmedicine.diseaseMitochondrial diseaseFanconi AnemiaPhenotypeClastogens030104 developmental biologylcsh:Biology (General)chemistryProoxidant stateCancer researchMitochondrial nutrientsMitochondrial dysfunctionlcsh:Medicine (General)030217 neurology & neurosurgeryHumanmedicine.drugRedox Biology
researchProduct

Whole Exome Sequencing Is the Preferred Strategy to Identify the Genetic Defect in Patients With a Probable or Possible Mitochondrial Cause

2018

Mitochondrial disorders, characterized by clinical symptoms and/or OXPHOS deficiencies, are caused by pathogenic variants in mitochondrial genes. However, pathogenic variants in some of these genes can lead to clinical manifestations which overlap with other neuromuscular diseases, which can be caused by pathogenic variants in non-mitochondrial genes as well. Mitochondrial pathogenic variants can be found in the mitochondrial DNA (mtDNA) or in any of the 1,500 nuclear genes with a mitochondrial function. We have performed a two-step next-generation sequencing approach in a cohort of 117 patients, mostly children, in whom a mitochondrial disease-cause could likely or possibly explain the phe…

0301 basic medicineMitochondrial DNANuclear genelcsh:QH426-470DISORDERSMitochondrial diseaseBiologyMOLECULAR DIAGNOSISMtDNA sequencingDNA sequencingDISEASEDiagnostic yield03 medical and health sciencesmedicineGeneticsDNA DELETIONSGenetics(clinical)whole-exome sequencingTRANSFER-RNA-SYNTHETASELACTIC-ACIDOSISGeneGenetics (clinical)Exome sequencingOriginal ResearchGeneticsmtDNA sequencingGenetic heterogeneityMUTATIONSASSEMBLY FACTORSmedicine.diseasePhenotypeMitochondrial diseaselcsh:Geneticsmitochondrial disease030104 developmental biologyHUMAN COMPLEX-IWhole-exome sequencingdiagnostic yieldNext-generation sequencingMolecular Medicinenext-generation sequencingLEIGH-SYNDROMEFrontiers in Genetics
researchProduct