Search results for "Mutation Types"

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Study of USH1 Splicing Variants through Minigenes and Transcript Analysis from Nasal Epithelial Cells

2012

Usher syndrome type I (USH1) is an autosomal recessive disorder characterized by congenital profound deafness, vestibular areflexia and prepubertal retinitis pigmentosa. The first purpose of this study was to determine the pathologic nature of eighteen USH1 putative splicing variants found in our series and their effect in the splicing process by minigene assays. These variants were selected according to bioinformatic analysis. The second aim was to analyze the USH1 transcripts, obtained from nasal epithelial cells samples of our patients, in order to corroborate the observed effect of mutations by minigenes in patient’s tissues. The last objective was to evaluate the nasal ciliary beat fre…

Usher syndromelcsh:Medicinemedicine.disease_causeGene SplicingMolecular cell biologyAutosomal Recessivelcsh:ScienceGeneticsMutationMultidisciplinaryCadherinsMyosin VIIaRNA splicingSensory PerceptionUsher SyndromesResearch ArticleRNA SplicingCadherin Related ProteinsBiologyMyosinsNoseGenetic MutationRetinitis pigmentosamedicineGeneticsotorhinolaryngologic diseasesHumansCiliaBiologyMessenger RNAlcsh:RIntronMutation TypesComputational BiologyGenetic VariationEpithelial CellsHuman Geneticsmedicine.diseaseMolecular biologyRNA processingMutagenesisCase-Control StudiesMutationGenetics of Diseaselcsh:QGene expressionSensory DeprivationPCDH15MinigeneCloningNeuroscience
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On the power and the systematic biases of the detection of chromosomal inversions by paired-end genome sequencing

2013

One of the most used techniques to study structural variation at a genome level is paired-end mapping (PEM). PEM has the advantage of being able to detect balanced events, such as inversions and translocations. However, inversions are still quite difficult to predict reliably, especially from high-throughput sequencing data. We simulated realistic PEM experiments with different combinations of read and library fragment lengths, including sequencing errors and meaningful base-qualities, to quantify and track down the origin of false positives and negatives along sequencing, mapping, and downstream analysis. We show that PEM is very appropriate to detect a wide range of inversions, even with …

Evolutionary GeneticsChromosome Structure and Functionlcsh:MedicineComputational biologyBiologyGenomeDNA sequencingStructural variation03 medical and health sciences0302 clinical medicineGenetic MutationGeneticsFalse positive paradoxHumansComputer SimulationFalse Positive ReactionsGenomic libraryGenome Sequencinglcsh:ScienceBiologyGenome EvolutionFalse Negative Reactions030304 developmental biologyChromosomal inversionSegmental duplicationGeneticsEvolutionary Biology0303 health sciencesMultidisciplinaryChromosome Biologylcsh:RBreakpointMutation TypesComputational BiologyChromosome MappingGenomic EvolutionGenomicsSequence Analysis DNAComparative GenomicsChromosomes Human Pair 1Chromosome Inversionlcsh:QStructural GenomicsSequence AnalysisAlgorithms030217 neurology & neurosurgeryResearch Article
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