Search results for "Frames"

showing 10 items of 265 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
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Mitochondrial introgression suggests extensive ancestral hybridization events among Saccharomyces species.

2017

Horizontal gene transfer (HGT) in eukaryotic plastids and mitochondrial genomes is common, and plays an important role in organism evolution. In yeasts, recent mitochondrial HGT has been suggested between S. cerevisiae and S. paradoxus. However, few strains have been explored given the lack of accurate mitochondrial genome annotations. Mitochondrial genome sequences are important to understand how frequent these introgressions occur, and their role in cytonuclear incompatibilities and fitness. Indeed, most of the Bateson-Dobzhansky-Muller genetic incompatibilities described in yeasts are driven by cytonuclear incompatibilities. We herein explored the mitochondrial inheritance of several wor…

0301 basic medicineMitochondrial DNAParadoxusGenomeSaccharomycesHoming endonucleaseElectron Transport Complex IV03 medical and health sciencesOpen Reading FramesSaccharomycesSpecies SpecificityGeneticsMolecular BiologyGeneEcology Evolution Behavior and SystematicsPhylogenybiologyBase SequenceGeographybiology.organism_classificationReticulate evolutionMitochondria030104 developmental biologyHaplotypesEvolutionary biologyHorizontal gene transferGenome Mitochondrialbiology.proteinHybridization GeneticSaccharomyces reticulate evolution mitochondrial introgression selfish elements recombination interspecies hybridizationSequence AlignmentMolecular phylogenetics and evolution
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The shared frameshift mutation landscape of microsatellite-unstable cancers suggests immunoediting during tumor evolution

2020

The immune system can recognize and attack cancer cells, especially those with a high load of mutation-induced neoantigens. Such neoantigens are abundant in DNA mismatch repair (MMR)-deficient, microsatellite-unstable (MSI) cancers. MMR deficiency leads to insertion/deletion (indel) mutations at coding microsatellites (cMS) and to neoantigen-inducing translational frameshifts. Here, we develop a tool to quantify frameshift mutations in MSI colorectal and endometrial cancer. Our results show that frameshift mutation frequency is negatively correlated to the predicted immunogenicity of the resulting peptides, suggesting counterselection of cell clones with highly immunogenic frameshift peptid…

0301 basic medicineMutation rateGeneral Physics and Astronomymedicine.disease_causeCOLORECTAL-CANCER0302 clinical medicineINDEL MutationMutation RateimmunologiaHLA AntigensNeoplasmsFrameshift Mutationlcsh:ScienceImmunologic SurveillanceGeneticsMutationMultidisciplinaryMISMATCH REPAIR DEFICIENCYQPEPTIDES3. Good healthkohdunrungon syöpäsyöpäsolutimmuunivaste030220 oncology & carcinogenesisTumour immunologyMicrosatellite InstabilityDNA mismatch repairINDEL MutationEXPRESSIONcongenital hereditary and neonatal diseases and abnormalitieskasvaimetDATABASESciencegastrointestinal cancerINSTABILITY3122 CancerssuolistosyövätBiologycomplex mixturesArticleGeneral Biochemistry Genetics and Molecular BiologyFrameshift mutationGastrointestinal cancer03 medical and health sciencesAntigens NeoplasmCOLONmedicineHumansCELLSelection GeneticIndelSIGNATUREStumour immunologyMicrosatellite instabilityGeneral ChemistryDNAmedicine.disease3126 Surgery anesthesiology intensive care radiologydigestive system diseases030104 developmental biologyImmunoeditinglcsh:Qmutaatiotbeta 2-MicroglobulinMicrosatellite Repeats
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Autosomal recessive truncatingMAB21L1mutation associated with a syndromic scrotal agenesis

2016

We report on a boy with a rare malformative association of scrotum agenesis, ophthalmological anomalies, cerebellar malformation, facial dysmorphism and global development delay. The reported patient was carrying a homozygous frameshift in MAB21L1 detected by whole-exome sequencing, considered as the most likely disease-causing variant. Mab21l1 knockout mice present a strikingly similar malformative association of ophthalmological malformations of the anterior chamber and preputial glands hypoplasia. We hypothesize that MAB21L1 haploinsufficiency cause a previously undescribed syndrome with scrotal agenesis, ophthalmological anomalies, facial dysmorphism and gross psychomotor delay as remar…

0301 basic medicinePathologymedicine.medical_specialtybusiness.industryPreputial gland030105 genetics & hereditymedicine.diseaseHypoplasiaFrameshift mutation03 medical and health sciences030104 developmental biologymedicine.anatomical_structureAgenesisScrotumGeneticsMedicinebusinessHaploinsufficiencyExomeGenetics (clinical)Exome sequencingClinical Genetics
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Dynamic evolution of mitochondrial genomes in Trebouxiophyceae, including the first completely assembled mtDNA from a lichen-symbiont microalga (Treb…

2019

AbstractTrebouxiophyceae (Chlorophyta) is a species-rich class of green algae with a remarkable morphological and ecological diversity. Currently, there are a few completely sequenced mitochondrial genomes (mtDNA) from diverse Trebouxiophyceae but none from lichen symbionts. Here, we report the mitochondrial genome sequence of Trebouxia sp. TR9 as the first complete mtDNA sequence available for a lichen-symbiont microalga. A comparative study of the mitochondrial genome of Trebouxia sp. TR9 with other chlorophytes showed important organizational changes, even between closely related taxa. The most remarkable change is the enlargement of the genome in certain Trebouxiophyceae, which is princ…

0301 basic medicinePrasiolalesTrebouxiaMitochondrial DNALichensEvolutionlcsh:MedicineBiologyDNA MitochondrialGenomeArticleEvolution MolecularOpen Reading Frames03 medical and health sciences0302 clinical medicineIntergenic regionSpecies SpecificityChlorophytaPhylogeneticsMicroalgaelcsh:SciencePhylogenyMultidisciplinaryTrebouxiophyceaelcsh:RSequence Analysis DNAGroup II intronbiology.organism_classificationIntrons030104 developmental biologyTandem Repeat SequencesEvolutionary biologyGenome Mitochondriallcsh:QPlant sciences030217 neurology & neurosurgeryScientific Reports
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Hot1 factor recruits co-activator Sub1 and elongation complex Spt4/5 to osmostress genes.

2016

Hyperosmotic stress response involves the adaptative mechanisms needed for cell survival. Under high osmolarity conditions, many stress response genes are activated by several unrelated transcription factors that are controlled by the Hog1 kinase. Osmostress transcription factor Hot1 regulates the expression of several genes involved in glycerol biosynthesis, and the presence of this transcription factor in their promoters is essential for RNApol II recruitment. The physical association between Hog1 and Hot1 activates this transcription factor and directs the RNA polymerase II localization at these promoters. We, herein, demonstrate that physical and genetic interactions exist between Hot1 …

0301 basic medicineSaccharomyces cerevisiae ProteinsChromosomal Proteins Non-HistoneResponse elementGenes FungalRNA polymerase IISaccharomyces cerevisiaeBiologyBiochemistry03 medical and health sciencesOpen Reading FramesOsmotic PressureRNA Processing Post-TranscriptionalPromoter Regions GeneticMolecular BiologyRNA polymerase II holoenzymeGeneticsGeneral transcription factorNuclear ProteinsPromoterCell BiologyDNA-Binding Proteins030104 developmental biologybiology.proteinTranscription factor II FTranscription factor II ETranscription factor II DTranscriptional Elongation FactorsProtein BindingTranscription FactorsThe Biochemical journal
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Norovirus GII.17 as Major Epidemic Strain in Italy, Winter 2015–16

2017

In winter 2015-16, norovirus GII.17 Kawasaki 2014 emerged as a cause of sporadic gastroenteritis in children in Italy. Median patient age was higher for those with GII.17 than GII.4 infection (55 vs. 24 months), suggesting limited cross-protection for older children.

0301 basic medicineSettore MED/07 - Microbiologia E Microbiologia ClinicaEpidemiologyviruseslcsh:Medicinemedicine.disease_causeDisease Outbreaksfluids and secretionsEpidemiologyChildEpidemic strainCaliciviridae InfectionsNorovirus GIIvirus diseasesInfectious DiseasesItalyChild PreschoolPopulation SurveillanceSeasonsgastroenteritigastroenteritisNorovirus GII.17 as Major Epidemic Strain in Italy Winter 2015–16Microbiology (medical)medicine.medical_specialtyAdolescentGenotype030106 microbiologyenteric infectionHistory 21st Centurylcsh:Infectious and parasitic diseasesOpen Reading Frames03 medical and health sciencesPatient ageResearch LettermedicineHumansviruseslcsh:RC109-216Noroviruvirusebusiness.industryenteric infectionsNoroviruslcsh:RInfant NewbornInfantGII.17 Kawasaki 2014Virology030104 developmental biologyNorovirusbusinessEmerging Infectious Diseases
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Temporal variation in the distribution of type-1 human astrovirus lineages in a settled population over 14 years.

2016

Human astroviruses (HAstVs) are important enteric pathogens that are genetically and antigenically heterogeneous and can be classified into eight sero/genotypes (HAstV-1 to -8) and different lineages within each HAstV type. This study describes the genetic diversity of HAstVs circulating in southern Italy over 14 years. Molecular analysis of HAstV-1 strains showed that three different lineages (1a, 1b and 1d) of the predominant genotype were circulating during the study period. The study of an archival collection of HAstV strains offers a unique opportunity to evaluate the patterns of variation of HAstV infections over the years and to correlate the observed epidemiological changes to the g…

0301 basic medicineSettore MED/07 - Microbiologia E Microbiologia Clinicamedicine.medical_specialtyTime FactorsGenotypePopulationGenome ViralBiology03 medical and health sciencesOpen Reading FramesMedical microbiologyVirologyAstroviridae InfectionsAstrovirus genotyping ItalyGenotypemedicineHumansGenetic variabilityeducationPhylogenyGeneticseducation.field_of_studyGenetic diversityMolecular EpidemiologyGenetic VariationGeneral MedicineHuman astrovirusMolecular analysis030104 developmental biologyItalyMamastrovirusArchives of virology
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Identification of transcribed protein coding sequence remnants within lincRNAs

2018

Abstract Long intergenic non-coding RNAs (lincRNAs) are non-coding transcripts >200 nucleotides long that do not overlap protein-coding sequences. Importantly, such elements are known to be tissue-specifically expressed and to play a widespread role in gene regulation across thousands of genomic loci. However, very little is known of the mechanisms for the evolutionary biogenesis of these RNA elements, especially given their poor conservation across species. It has been proposed that lincRNAs might arise from pseudogenes. To test this systematically, we developed a novel method that searches for remnants of protein-coding sequences within lincRNA transcripts; the hypothesis is that we can t…

0301 basic medicineTransposable elementSequence analysisPseudogeneRetrotransposonComputational biologyBiologyOpen Reading Frames03 medical and health sciences0302 clinical medicineIntergenic regionSequence Analysis ProteinGeneticsHumansAmino Acid SequenceGeneRegulation of gene expressionBase SequenceSequence Analysis RNAComputational Biology030104 developmental biologyGene Expression RegulationDNA IntergenicRNA Long NoncodingSequence AlignmentAlgorithms030217 neurology & neurosurgeryBiogenesisNucleic Acids Research
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Frame-related Sequences in Chains and Scales of Hilbert Spaces

2022

Frames for Hilbert spaces are interesting for mathematicians but also important for applications in, e.g., signal analysis and physics. In both mathematics and physics, it is natural to consider a full scale of spaces, and not only a single one. In this paper, we study how certain frame-related properties of a certain sequence in one of the spaces, such as completeness or the property of being a (semi-) frame, propagate to the other ones in a scale of Hilbert spaces. We link that to the properties of the respective frame-related operators, such as analysis or synthesis. We start with a detailed survey of the theory of Hilbert chains. Using a canonical isomorphism, the properties of frame se…

42C15 46C99 47A70Algebra and Number TheoryHilbert chainsLogicFunctional Analysis (math.FA)Mathematics - Functional AnalysisSettore MAT/05 - Analisi Matematicaframes; scales of Hilbert spaces; Hilbert chains; Bessel sequences; semi-framesframesFOS: Mathematicsscales of Hilbert spacessemi-framesGeometry and TopologyBessel sequencesMathematical PhysicsAnalysis
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