2025
The RNA-binding protein TRIM71 is essential for hearing in humans and mice and times auditory sensory organ development.
Li X, Morgan C, Duy P, Evsen L, Hao L, Machavoine R, Belhous K, Ernest S, Denoyelle F, Mignot C, Brioude F, Parodi M, Li L, Huang H, Nadar Ponniah P, Kolanus W, Kahle K, Marlin S, Doetzlhofer A. The RNA-binding protein TRIM71 is essential for hearing in humans and mice and times auditory sensory organ development. Proceedings Of The National Academy Of Sciences Of The United States Of America 2025, 122: e2505811122. PMID: 40892928, PMCID: PMC12435228, DOI: 10.1073/pnas.2505811122.Peer-Reviewed Original ResearchConceptsKnockout miceAssociated with hearing lossMonoallelic missense mutationsTiming of cell cycle exitHair cellsCochlear progenitor cellsSensory organ developmentInner ear morphogenesisStereotypic timeCell cycle exitOuter hair cell regionHair cell regionMechanosensory hair cellsSevere HLHair cell progenitorsHearing deficitsEarly otic developmentProgenitor cellsAuditory sensory organCell progenitorsEar morphogenesisGenetic studiesMissense mutationsCycle exitRisk genes
2016
Innervation regulates synaptic ribbons in lateral line mechanosensory hair cells
Suli A, Pujol R, Cunningham DE, Hailey DW, Prendergast A, Rubel EW, Raible DW. Innervation regulates synaptic ribbons in lateral line mechanosensory hair cells. Journal Of Cell Science 2016, 129: 2250-2260. PMID: 27103160, PMCID: PMC4920245, DOI: 10.1242/jcs.182592.Peer-Reviewed Original ResearchConceptsMechanosensory hair cellsHair cellsWild-type larvaeZebrafish lateral line systemElectron-dense structuresRibbon formationProper synapsisLateral line systemMature synapseSynaptic vesiclesHair cell innervationPost-synaptic elementsSensory cellsBasolateral membranePresynaptic zoneAfferent fibersSynaptic ribbonsFunctional synapsesBalance disordersCellsInnervationMutantsSynapsesLarvaeCytoplasm
2001
α10: A determinant of nicotinic cholinergic receptor function in mammalian vestibular and cochlear mechanosensory hair cells
Elgoyhen A, Vetter D, Katz E, Rothlin C, Heinemann S, Boulter J. α10: A determinant of nicotinic cholinergic receptor function in mammalian vestibular and cochlear mechanosensory hair cells. Proceedings Of The National Academy Of Sciences Of The United States Of America 2001, 98: 3501-3506. PMID: 11248107, PMCID: PMC30682, DOI: 10.1073/pnas.051622798.Peer-Reviewed Original ResearchConceptsHair cellsMechanosensory hair cellsCholinergic receptor functionAgonist-mediated desensitizationHair cell functionAlpha9 nAChREfferent modulationHeteromeric nAChRsAlpha9alpha10 nAChRsPharmacological profileBiphasic responseMammalian vestibularReceptor functionAlpha10 subunitsNAChRsCell functionAlpha9Xenopus laevis oocytesCurrent-voltage relationshipVertebrate hair cellsSubunit geneLaevis oocytesAlpha10CellsAlpha9alpha10
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