Search results for "Otic vesicle"

showing 3 items of 3 documents

Novel form of X-linked nonsyndromic hearing loss with cochlear malformation caused by a mutation in the type IV collagen gene COL4A6

2013

Hereditary hearing loss is the most common human sensorineural disorder. Genetic causes are highly heterogeneous, with mutations detected in >40 genes associated with nonsyndromic hearing loss, to date. Whereas autosomal recessive and autosomal dominant inheritance is prevalent, X-linked forms of nonsyndromic hearing impairment are extremely rare. Here, we present a Hungarian three-generation family with X-linked nonsyndromic congenital hearing loss and the underlying genetic defect. Next-generation sequencing and subsequent segregation analysis detected a missense mutation (c.1771G>A, p.Gly591Ser) in the type IV collagen gene COL4A6 in all affected family members. Bioinformatic analysis an…

Collagen Type IVMaleHearing lossDNA Mutational AnalysisMolecular Sequence DataMutation MissenseGene ExpressionDeafnessBiologyCongenital hearing lossmedicine.disease_causeArticleType IV collagenotorhinolaryngologic diseasesGeneticsmedicineAnimalsHumansMissense mutationGenetic Predisposition to DiseaseAmino Acid SequenceAlport syndromeGeneCells CulturedGenetic Association StudiesZebrafishGenetics (clinical)GeneticsMutationGenetic Diseases X-LinkedMiddle Agedmedicine.diseaseCochleaPedigreeMice Inbred C57BLChild PreschoolFemaleRNA Splice SitesOtic vesiclemedicine.symptomEuropean Journal of Human Genetics
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Expression analysis of jagged genes in zebrafish embryos

2005

The interaction of transmembrane Delta and Jagged/Serrate ligands with Notch receptors on neighboring cells is critically involved in cell specification during development. In zebrafish, the early expression of delta but not of jagged genes has been investigated in some detail. We have analyzed the sequence and embryonic expression pattern of the three zebrafish genes jagged1a, jagged1b, and jagged2. These genes, whose transcripts are detectable by in situ hybridization from early somitogenesis, are widely and dynamically expressed in embryos. Coexpression is limited, however, to the notochord and lens (jagged1a and jagged1b) and to the otic vesicle and pronephros (jagged1b and jagged2). Co…

Nervous systemanimal structuresNotchNotch signaling pathwayNotochordBiologystomatognathic systemSomitogenesisNotochordmedicineAnimalsPancreaSerrate-Jagged ProteinsSomitePlacodeZebrafishPhylogenyNotch signalingZebrafishGeneticsVertebrateCalcium-Binding ProteinsGene Expression Regulation DevelopmentalMembrane ProteinsCell BiologyZebrafish Proteinsbiology.organism_classificationCell biologyPronephrosmedicine.anatomical_structurezebrafish; Notch; JaggedEmbryoIntercellular Signaling Peptides and ProteinsPronephroOtic vesicleJaggedJagged-2 ProteinOtic PlacodesDevelopmental biologyIn situ hybridizationJagged/serrate geneEmbryo; In situ hybridization; Jagged/serrate genes; Nervous system; Notch signaling; Notochord; Pancreas; Placodes; Pronephros; Somites; Vertebrate; Zebrafish; Developmental Biology; Cell BiologyDevelopmental Biology
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In silico characterization of an Iroquois family-related homeodomain protein.

2005

Homeobox genes have been demonstrated to play important roles during cancer differentiation and embryonic development. The subset of Iroquois-related homeobox genes (IRXs) have furthermore been. demonstrated to be involved in several embryonic developmental processes such as patterning of the anterior-posterior and dorso-ventral axis, as well as specific regions of the central nervous system, and differentiation of the otic vesicle, branchial epithelium, and limbs. We have characterized a novel homeodomain protein and corresponding gene by means of computational biology. Since the protein sequence displayed high similarity to the human IRX proteins, the newly identified homeodomain protein …

TBX1EMX2Molecular Sequence DataHomeobox A1BiologyHomeobox protein Nkx-2.5NKX2-3MiceGene OrderGeneticsAnimalsHumansAmino Acid SequenceRNA MessengerPhylogenyZebrafishExpressed Sequence TagsHomeodomain ProteinsBase SequenceGene Expression ProfilingChromosome MappingComputational BiologyGeneral MedicineExonsZebrafish ProteinsMolecular biologyIntronsGenesPAX4HomeoboxOtic vesicleTranscription FactorsInternational journal of molecular medicine
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