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  • synaptic
  • We show that a cage-shaped F-actin network is essential for maintaining a tight spatial organization of Cav1.3 Ca(2+) channels at the synaptic ribbons of auditory inner hair cells. (pasteur.fr)
  • We propose a new mechanistic model for vesicle exocytosis in auditory hair cells where the rate of vesicle recruitment to the ribbons is directly controlled by a synaptic F-actin network and changes in intracellular hydrostatic pressure. (pasteur.fr)
  • Given that any deficits in synaptic transmission at the hair cell afferent fiber synapse results in auditory deficits, identifying novel mechanisms may provide unique avenues for intervention. (grantome.com)
  • IHCs, the primary sensory cells of the inner ear, make synaptic contact with VIIIth nerve afferents and are responsible for the transmission of the signal to the central nervous system (CNS). (oxfordre.com)
  • Synaptic vesicle recycling sustains high rates of neurotransmission at the ribbon-type active zones (AZs) of mouse auditory inner hair cells (IHCs), but its modes and molecular regulation are poorly understood. (bfnt-goettingen.de)
  • Otoferlin interacts with myosin VI: implications for maintenance of the basolateral synaptic structure of the inner hair cell. (semanticscholar.org)
  • Complementing mammalian studies, zebrafish have been used to gain significant insights into many facets of hair cell biology, including mechanotransduction and synaptic physiology as well as mechanisms of both hereditary and acquired hair cell dysfunction. (springer.com)
  • SIGNIFICANCE STATEMENT Inner hair cells possess calcium channels that are essential for transmitting sound information into synaptic transmitter release. (jneurosci.org)
  • IHCs
  • One row of inner hair cells (IHCs) is present on the modiolar side of the organ, and there are three to four rows of adjacent outer hair cells (OHCs). (oxfordre.com)
  • Both IHCs and OHCs are mechanosensory cells that detect sound pressure waves transmitted along the auditory organ. (oxfordre.com)
  • Peak Ca 2+ and Ba 2+ currents of mature du/du inner hair cells (IHCs) were reduced by 30-40%, respectively, and gating properties, such as the voltage of half-maximum activation and voltage sensitivity, were altered, indicating that Ca v 1.3 channels normally coassemble with α 2 δ2 at IHC presynapses. (jneurosci.org)
  • 2018
  • Becker L, Schnee ME, Niwa M et al (2018) The presynaptic ribbon maintains vesicle populations at the hair cell afferent fiber synapse. (springer.com)
  • Blanco-Sánchez B, Clément A, Fierro J Jr, Stednitz S, Phillips JB, Wegner J, Panlilio JM, Peirce JL, Washbourne P, Westerfield M (2018) Grxcr1 promotes hair bundle development by destabilizing the physical interaction between harmonin and Sans Usher syndrome proteins. (springer.com)
  • Mice
  • This essential role of CIB2 in mechanotransduction and cell survival that, we show, is restricted to the cochlea, probably accounts for the presence in CIB2 -/- mice and CIB2 patients, unlike in Usher syndrome, of isolated hearing loss without balance and vision deficits. (nih.gov)
  • Early postnatal development of spontaneous and acoustically evoked discharge activity of principal cells of the medial nucleus of the trapezoid body: an in vivo study in mice. (semanticscholar.org)
  • This is based on recent studies showing that supporting cells purified from pre-hearing neonatal mice or 2 week old hearing mice have the capacity to switch cell fate and trans-differentiate into hair cells - . (pubmedcentralcanada.ca)
  • The mutant mice displayed an elevation of auditory brianstem response (ABR) thresholds responsing to click and tones of different frequencies, especially high frequency, in the similar pattern of human's cilinic phenotype. (egms.de)
  • cochlea
  • However, it is unresolved how Notch signaling functions in the hair cell-damaged cochlea and the molecular and cellular changes induced in supporting cells in response to hair cell trauma are poorly understood. (pubmedcentralcanada.ca)
  • Moreover, we provide evidence that Notch signaling is active in the hair cell damaged cochlea and identify Hes1, Hey1, Hey2, HeyL, and Sox2 as targets and potential Notch effectors of this hair cell-independent mechanism of Notch signaling. (pubmedcentralcanada.ca)
  • We set out to determine the outcome of overexpression of Math1 in nonsensory cells of the cochlea on the phenotype of these cells. (jneurosci.org)
  • exocytosis
  • Using a mouse model with a mutant, nonfunctional α 2 δ2 protein, we showed that the α 2 δ2 protein is necessary for normal calcium currents and exocytosis in inner hair cells. (jneurosci.org)
  • 1978
  • Bagger-Sjöbäck D, Wersäll J (1978) Gentamicin-induced mitochondrial damage in inner ear sensory cells of the lizard Calotes versicolor. (springer.com)
  • system
  • All of the signal processing that happens at the auditory periphery is communicated to the central nervous system through the hair cell afferent fiber synapse. (grantome.com)
  • Although perhaps best known for their use in developmental studies, over the last couple of decades, zebrafish have become increasingly popular model organisms for investigating auditory system function and disease. (springer.com)
  • Briefly describe how different elements of the anatomy of the auditory system are involved in sound perception? (brainscape.com)
  • Third, the role of the auditory efferent system, which receives inputs from L/MSR fibers, is revisited in the context of fluctuation-profile coding. (springer.com)
  • The auditory efferent system is hypothesized to maintain and enhance neural fluctuation profiles. (springer.com)
  • bundle
  • All these organs have a common receptor cell type, which is called the hair cell , for the bundle of enlarged microvilli protruding from its apical surface. (oxfordre.com)
  • The proximal stimulus for all hair cells is deflection of the mechanosensitive hair bundle. (oxfordre.com)
  • Hair cells convert mechanical information contained within the temporal pattern of hair bundle deflections into electrical signals, which they transmit to the brain for interpretation. (oxfordre.com)
  • By contrast, OHCs are signal amplifiers that generate force using active hair-bundle motility and somatic electromotility, the latter in response to depolarization that follows hair bundle displacement. (oxfordre.com)
  • C: Transmission electron micrograph of a P2 + 1 day in culture hair bundle from a mouse OHC. (oxfordre.com)
  • Developmental
  • In an effort to find therapeutic targets for deafness and balance disorders, scientists have examined gene expression patterns in auditory tissues under different developmental and experimental conditions. (mdpi.com)
  • development and survival
  • In a study published last year, she and her colleagues identified a new role for a particular group of proteins, known as RFX transcription factors, in the development and survival of the hair cells. (umaryland.edu)
  • Immature
  • Immature mouse cochleae and UB/OC-1 cells were used in this work to address whether specific variants of PCDH15 and VLGR1 are being selectively transported to opposite poles of the hair cells. (biomedsearch.com)
  • Moreover, we show that these hair cell-like cells, generated by supporting cells have molecular, cellular, and basic electrophysiological properties similar to immature hair cells rather than supporting cells. (pubmedcentralcanada.ca)
  • Overexpression of Math1 in cultures of immature rat cochleae results in the production of ectopic hair cells derived from nonsensory epithelial precursors ( Zheng and Gao, 2000 ). (jneurosci.org)
  • thresholds
  • A functional α 2 δ2-null mouse, the ducky mouse (du), showed elevated auditory brainstem response click and frequency-dependent hearing thresholds. (jneurosci.org)
  • We found that hair cells of the mouse express the auxiliary subunit α 2 δ2, which is needed for normal hearing thresholds. (jneurosci.org)
  • proliferation
  • Cell proliferation was quantified through BrdU labeling at 0, 1, 2, 3, 7 and 10 days after the end of sound exposure. (biomedcentral.com)
  • Cell proliferation peaked two days post-noise exposure in the caudal region, and to a lesser extent in the rostral region. (biomedcentral.com)
  • Cellular renewal on the basis of stem (basal) cell proliferation is a hallmark of most epithelial tissues. (jneurosci.org)
  • mechanotransduction
  • Whole-cell patch-clamp recordings demonstrate that these transient PCDH15/PCDH15 links mediate mechanotransduction currents of normal amplitude but abnormal Ca(2+)-dependent decay (adaptation). (nih.gov)
  • CIB2, defective in isolated deafness, is key for auditory hair cell mechanotransduction and survival. (nih.gov)
  • Hearing Loss
  • Loss of these cells in humans leads to permanent hearing loss, since mammalian hair cells cannot be regenerated. (emory.edu)
  • Eventually, she says, it might be possible to use our increased understanding of RFX transcription factor to treat hearing loss, by either protecting hair cells from death or fostering their growth. (umaryland.edu)
  • Though the hypoxia did not cause hearing loss and hair cell loss, the combined exposure to noise and hypoxic air caused more hearing loss and hair cell loss than the noise alone. (biomedsearch.com)
  • calcium
  • To ensure that the ears can detect even the softest sounds, the tip-links always have a small amount of tension which allows a small, but continuous flow of calcium ions into the cell. (elifesciences.org)
  • apical
  • Tight junctions that ring the apical surfaces of hair cells and supporting cells separate the endolymph from the perilymph , a fluid similar to normal extracellular solution that bathes the basolateral surfaces of hair cells. (oxfordre.com)