Delayed structural maturation of inner hair cell ribbon synapses in a mouse model of fragile X syndrome [PDF]
Clinical features of the fragile X syndrome (FXS) phenotype include intellectual disability, repetitive behaviors, social communication deficits, and, commonly, auditory hypersensitivity to acoustic stimuli.
M. Chojnacka +2 more
doaj +2 more sources
Mechanoelectrical transducer (MET) channels on the tips of inner hair cells are essential for transducing auditory sensory information. Here, the authors show that disrupting MET channel function also prevents the preservation of normal inner hair cell ...
Laura F. Corns +12 more
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Connexin-Mediated Signaling in Nonsensory Cells Is Crucial for the Development of Sensory Inner Hair Cells in the Mouse Cochlea [PDF]
Mutations in the genes encoding for gap junction proteins connexin 26 (Cx26) and connexin 30 (Cx30) have been linked to syndromic and nonsyndromic hearing loss in mice and humans. The release of ATP from connexin hemichannels in cochlear nonsensory cells
Stuart L. Johnson +8 more
semanticscholar +3 more sources
Severe hearing loss and outer hair cell death in homozygous Foxo3 knockout mice after moderate noise exposure [PDF]
Noise induced hearing loss (NIHL) is a disease that affects millions of Americans. Identifying genetic pathways that influence recovery from noise exposure is an important step forward in understanding NIHL.
Felicia Gilels +4 more
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Conserved and Divergent Principles of Planar Polarity Revealed by Hair Cell Development and Function
Planar polarity describes the organization and orientation of polarized cells or cellular structures within the plane of an epithelium. The sensory receptor hair cells of the vertebrate inner ear have been recognized as a preeminent vertebrate model ...
Michael R. Deans, Michael R. Deans
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IntroductionThe inner ear hair cells of fishes can provide insight into the early evolution of vertebrate inner ear structure. Fishes represent some of the first vertebrates to evolve auditory capacity, and the same basic structure, the sensory hair cell,
Derek J. Sauer +2 more
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Non-mammalian vertebrates can restore their auditory and vestibular hair cells naturally by triggering the regeneration of adjacent supporting cells. The transcription factor ATOH1 is a key regulator of hair cell development and regeneration in the inner
Amrita A. Iyer, A. Groves
semanticscholar +1 more source
Mapping the regulatory landscape of auditory hair cells from single-cell multi-omics data
Auditory hair cells transduce sound to the brain, and in mammals, these cells reside together with supporting cells in the sensory epithelium of the cochlea, called the organ of Corti.
Shuze Wang +4 more
semanticscholar +1 more source
An antibody to RGMa promotes regeneration of cochlear synapses after noise exposure
Auditory neuropathy is caused by the loss of afferent input to the brainstem via the components of the neural pathway comprising inner hair cells and the first order neurons of the spiral ganglion.
Jerome Nevoux +8 more
doaj +1 more source
Aminoglycoside-induced hair cell death of inner ear organs causes functional deficits in adult zebrafish (Danio rerio). [PDF]
Aminoglycoside antibiotics, like gentamicin, kill inner ear sensory hair cells in a variety of species including chickens, mice, and humans. The zebrafish (Danio rerio) has been used to study hair cell cytotoxicity in the lateral line organs of larval ...
Phillip M Uribe +8 more
doaj +1 more source

