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http://purl.uniprot.org/citations/34544869http://www.w3.org/1999/02/22-rdf-syntax-ns#typehttp://purl.uniprot.org/core/Journal_Citation
http://purl.uniprot.org/citations/34544869http://www.w3.org/2000/01/rdf-schema#comment"Mutations in the gene for Norrie disease protein (Ndp) cause syndromic deafness and blindness. We show here that cochlear function in an Ndp knockout mouse deteriorated with age: At P3-P4, hair cells (HCs) showed progressive loss of Pou4f3 and Gfi1, key transcription factors for HC maturation, and Myo7a, a specialized myosin required for normal function of HC stereocilia. Loss of expression of these genes correlated to increasing HC loss and profound hearing loss by 2 mo. We show that overexpression of the Ndp gene in neonatal supporting cells or, remarkably, up-regulation of canonical Wnt signaling in HCs rescued HCs and cochlear function. We conclude that Ndp secreted from supporting cells orchestrates a transcriptional network for the maintenance and survival of HCs and that increasing the level of β-catenin, the intracellular effector of Wnt signaling, is sufficient to replace the functional requirement for Ndp in the cochlea."xsd:string
http://purl.uniprot.org/citations/34544869http://purl.org/dc/terms/identifier"doi:10.1073/pnas.2106369118"xsd:string
http://purl.uniprot.org/citations/34544869http://purl.uniprot.org/core/author"Hayashi Y."xsd:string
http://purl.uniprot.org/citations/34544869http://purl.uniprot.org/core/author"Tian C."xsd:string
http://purl.uniprot.org/citations/34544869http://purl.uniprot.org/core/author"Indzhykulian A.A."xsd:string
http://purl.uniprot.org/citations/34544869http://purl.uniprot.org/core/author"Chiang H."xsd:string
http://purl.uniprot.org/citations/34544869http://purl.uniprot.org/core/author"Edge A.S.B."xsd:string
http://purl.uniprot.org/citations/34544869http://purl.uniprot.org/core/date"2021"xsd:gYear
http://purl.uniprot.org/citations/34544869http://purl.uniprot.org/core/name"Proc Natl Acad Sci U S A"xsd:string
http://purl.uniprot.org/citations/34544869http://purl.uniprot.org/core/pages"e2106369118"xsd:string
http://purl.uniprot.org/citations/34544869http://purl.uniprot.org/core/title"Norrie disease protein is essential for cochlear hair cell maturation."xsd:string
http://purl.uniprot.org/citations/34544869http://purl.uniprot.org/core/volume"118"xsd:string
http://purl.uniprot.org/citations/34544869http://www.w3.org/2004/02/skos/core#exactMatchhttp://purl.uniprot.org/pubmed/34544869
http://purl.uniprot.org/citations/34544869http://xmlns.com/foaf/0.1/primaryTopicOfhttps://pubmed.ncbi.nlm.nih.gov/34544869
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http://purl.uniprot.org/uniprot/#_A0A0U1RPX7-mappedCitation-34544869http://www.w3.org/1999/02/22-rdf-syntax-ns#objecthttp://purl.uniprot.org/citations/34544869
http://purl.uniprot.org/uniprot/#_F7CRC6-mappedCitation-34544869http://www.w3.org/1999/02/22-rdf-syntax-ns#objecthttp://purl.uniprot.org/citations/34544869