Stuart L. Johnson
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View article: Aging Alters Hair Cell Physiological Properties in Mice with Late-Onset Age-Related Hearing Loss
Aging Alters Hair Cell Physiological Properties in Mice with Late-Onset Age-Related Hearing Loss Open
Cochlear hair cells convert sound stimuli into electrical signals. Age-related hearing loss (ARHL) is characterized by the progressive reduction of these hair cells and their synapses, but the underlying mechanisms remain poorly understood…
View article: Adeno‐associated virus‐based rescue of <i>Myo7a</i> expression restores hair‐cell function and improves hearing thresholds in a <i>USH1B</i> mouse strain
Adeno‐associated virus‐based rescue of <i>Myo7a</i> expression restores hair‐cell function and improves hearing thresholds in a <i>USH1B</i> mouse strain Open
Mutations in MYO7A , the gene encoding the unconventional myosin 7a, cause hereditary deafness in mice and humans. In the cochlea, MYO7A is present in the sensory hair cells from embryonic stages of development, and plays a critical role i…
View article: Transient capacitance changes recorded from vestibular type I hair cells are produced by <i>G</i><sub>K,L</sub> gating and do not involve neurotransmitter exocytosis
Transient capacitance changes recorded from vestibular type I hair cells are produced by <i>G</i><sub>K,L</sub> gating and do not involve neurotransmitter exocytosis Open
Head movements are detected and signalled to primary sensory neurons by vestibular types I and II hair cells. Signal transmission involves glutamate exocytosis from hair cells, which is triggered by Ca 2+ inflow through voltage‐gated Ca V …
View article: MYO7A is required for the functional integrity of the mechanoelectrical transduction complex in hair cells of the adult cochlea
MYO7A is required for the functional integrity of the mechanoelectrical transduction complex in hair cells of the adult cochlea Open
Myosin-VIIA (MYO7A) is an unconventional myosin responsible for syndromic (Usher 1B) or nonsyndromic forms of deafness in humans when mutated. In the cochlea, MYO7A is expressed in hair cells, where it is believed to act as the motor prote…
View article: In vivo AAV9-Myo7a gene rescue restores hearing and cholinergic efferent innervation in inner hair cells
In vivo AAV9-Myo7a gene rescue restores hearing and cholinergic efferent innervation in inner hair cells Open
In the mammalian cochlea, sensory hair cells are crucial for the transduction of acoustic stimuli into electrical signals, which are then relayed to the central auditory pathway via spiral ganglion neuron (SGN) afferent dendrites. The SGN …
View article: The upregulation of K<sup>+</sup> and HCN channels in developing spiral ganglion neurons is mediated by cochlear inner hair cells
The upregulation of K<sup>+</sup> and HCN channels in developing spiral ganglion neurons is mediated by cochlear inner hair cells Open
Spiral ganglion neurons (SGNs) are primary sensory afferent neurons that relay acoustic information from the cochlear inner hair cells (IHCs) to the brainstem. The response properties of different SGNs diverge to represent a wide range of …
View article: Pleiotropic brain function of whirlin identified by a novel mutation
Pleiotropic brain function of whirlin identified by a novel mutation Open
View article: BAI1 localizes AMPA receptors at the cochlear afferent post-synaptic density and is essential for hearing
BAI1 localizes AMPA receptors at the cochlear afferent post-synaptic density and is essential for hearing Open
Type I spiral ganglion neurons (SGNs) convey sound information to the central auditory pathway by forming synapses with inner hair cells (IHCs) in the mammalian cochlea. The molecular mechanisms regulating the formation of the post-synapti…
View article: A critical period of prehearing spontaneous Ca <sup>2+</sup> spiking is required for hair‐bundle maintenance in inner hair cells
A critical period of prehearing spontaneous Ca <sup>2+</sup> spiking is required for hair‐bundle maintenance in inner hair cells Open
View article: AAV-mediated rescue of Eps8 expression in vivo restores hair-cell function in a mouse model of recessive deafness
AAV-mediated rescue of Eps8 expression in vivo restores hair-cell function in a mouse model of recessive deafness Open
View article: Signal transmission in mature mammalian vestibular hair cells
Signal transmission in mature mammalian vestibular hair cells Open
The maintenance of balance and gaze relies on the faithful and rapid signaling of head movements to the brain. In mammals, vestibular organs contain two types of sensory hair cells, type-I and type-II, which convert the head motion-induced…
View article: HDAC6 inhibition partially alleviates mitochondrial trafficking defects and restores motor function in human motor neuron and zebrafish models of Charcot-Marie-Tooth Disease Type 2A
HDAC6 inhibition partially alleviates mitochondrial trafficking defects and restores motor function in human motor neuron and zebrafish models of Charcot-Marie-Tooth Disease Type 2A Open
Charcot Marie Tooth Disease (CMT) is a group of inherited progressive conditions affecting distal motor and sensory neurons, leading to muscle weakness, pain and loss of sensation in limbs. There are currently no treatments for this debili…
View article: Grxcr1 regulates hair bundle morphogenesis and is required for normal mechanoelectrical transduction in mouse cochlear hair cells
Grxcr1 regulates hair bundle morphogenesis and is required for normal mechanoelectrical transduction in mouse cochlear hair cells Open
Tasmanian devil ( tde ) mice are deaf and exhibit circling behaviour. Sensory hair cells of mutants show disorganised hair bundles with abnormally thin stereocilia. The origin of this mutation is the insertion of a transgene which disrupts…
View article: Current Response in CaV1.3–/– Mouse Vestibular and Cochlear Hair Cells
Current Response in CaV1.3–/– Mouse Vestibular and Cochlear Hair Cells Open
Signal transmission by sensory auditory and vestibular hair cells relies upon Ca 2+ -dependent exocytosis of glutamate. The Ca 2+ current in mammalian inner ear hair cells is predominantly carried through Ca V 1.3 voltage-gated Ca 2+ chann…
View article: MET currents and otoacoustic emissions from mice with a detached tectorial membrane indicate the extracellular matrix regulates Ca<sup>2+</sup> near stereocilia
MET currents and otoacoustic emissions from mice with a detached tectorial membrane indicate the extracellular matrix regulates Ca<sup>2+</sup> near stereocilia Open
Key points The aim was to determine whether detachment of the tectorial membrane (TM) from the organ of Corti in Tecta/Tectb −/− mice affects the biophysical properties of cochlear outer hair cells (OHCs). Tecta/Tectb −/− mice have highly …
View article: Biophysical and morphological changes in inner hair cells and their efferent innervation in the ageing mouse cochlea
Biophysical and morphological changes in inner hair cells and their efferent innervation in the ageing mouse cochlea Open
Key points Age‐related hearing loss is a progressive hearing loss involving environmental and genetic factors, leading to a decrease in hearing sensitivity, threshold and speech discrimination. We compared age‐related changes in inner hair…
View article: Pathophysiological changes in inner hair cell ribbon synapses in the ageing mammalian cochlea
Pathophysiological changes in inner hair cell ribbon synapses in the ageing mammalian cochlea Open
Key points Age‐related hearing loss (ARHL) is associated with the loss of inner hair cell (IHC) ribbon synapses, lower hearing sensitivity and decreased ability to understand speech, especially in a noisy environment. Little is known about…
View article: Age‐related changes in the biophysical and morphological characteristics of mouse cochlear outer hair cells
Age‐related changes in the biophysical and morphological characteristics of mouse cochlear outer hair cells Open
Key points Age‐related hearing loss (ARHL) is a very heterogeneous disease, resulting from cellular senescence, genetic predisposition and environmental factors (e.g. noise exposure). Currently, we know very little about age‐related change…
View article: Exocytosis in mouse vestibular Type II hair cells shows a high‐order Ca <sup>2+</sup> dependence that is independent of synaptotagmin‐4
Exocytosis in mouse vestibular Type II hair cells shows a high‐order Ca <sup>2+</sup> dependence that is independent of synaptotagmin‐4 Open
Mature hair cells transduce information over a wide range of stimulus intensities and frequencies for prolonged periods of time. The efficiency of such a demanding task is reflected in the characteristics of exocytosis at their specialized…
View article: K+ Accumulation and Clearance in the Calyx Synaptic Cleft of Type I Mouse Vestibular Hair Cells
K+ Accumulation and Clearance in the Calyx Synaptic Cleft of Type I Mouse Vestibular Hair Cells Open
Vestibular organs of Amniotes contain two types of sensory cells, named Type I and Type II hair cells. While Type II hair cells are contacted by several small bouton nerve terminals, Type I hair cells receive a giant terminal, called a cal…
View article: Hair cell maturation is differentially regulated along the tonotopic axis of the mammalian cochlea
Hair cell maturation is differentially regulated along the tonotopic axis of the mammalian cochlea Open
Key points Outer hair cells (OHCs) enhance the sensitivity and the frequency tuning of the mammalian cochlea. Similar to the primary sensory receptor, the inner hair cells (IHCs), the mature functional characteristics of OHCs are acquired …
View article: Clarin‐2 is essential for hearing by maintaining stereocilia integrity and function
Clarin‐2 is essential for hearing by maintaining stereocilia integrity and function Open
Hearing relies on mechanically gated ion channels present in the actin-rich stereocilia bundles at the apical surface of cochlear hair cells. Our knowledge of the mechanisms underlying the formation and maintenance of the sound-receptive s…
View article: Dynamic coupling of cochlear inner hair cell intrinsic Ca<sup>2+</sup>action potentials to Ca<sup>2+</sup>signaling of non-sensory cells
Dynamic coupling of cochlear inner hair cell intrinsic Ca<sup>2+</sup>action potentials to Ca<sup>2+</sup>signaling of non-sensory cells Open
The relationship between Ca 2+ action potential (AP) activity in immature inner hair cells (IHCs) and the spontaneous ATP-dependent intercellular Ca 2+ signaling in cochlear non-sensory cells (NSCs) of the greater epithelial ridge (GER) is…
View article: Gata3 is required for the functional maturation of inner hair cells and their innervation in the mouse cochlea
Gata3 is required for the functional maturation of inner hair cells and their innervation in the mouse cochlea Open
Key points The physiological maturation of auditory hair cells and their innervation requires precise temporal and spatial control of cell differentiation. The transcription factor gata3 is essential for the earliest stages of auditory sys…
View article: Coordinated calcium signalling in cochlear sensory and non‐sensory cells refines afferent innervation of outer hair cells
Coordinated calcium signalling in cochlear sensory and non‐sensory cells refines afferent innervation of outer hair cells Open
View article: Hair Cell Afferent Synapses: Function and Dysfunction
Hair Cell Afferent Synapses: Function and Dysfunction Open
To provide a meaningful representation of the auditory landscape, mammalian cochlear hair cells are optimized to detect sounds over an incredibly broad range of frequencies and intensities with unparalleled accuracy. This ability is largel…
View article: Helios is a key transcriptional regulator of outer hair cell maturation
Helios is a key transcriptional regulator of outer hair cell maturation Open
View article: Mechanotransduction is required for establishing and maintaining mature inner hair cells and regulating efferent innervation
Mechanotransduction is required for establishing and maintaining mature inner hair cells and regulating efferent innervation Open
In the adult auditory organ, mechanoelectrical transducer (MET) channels are essential for transducing acoustic stimuli into electrical signals. In the absence of incoming sound, a fraction of the MET channels on top of the sensory hair ce…
View article: Human axial progenitors generate trunk neural crest cells in vitro
Human axial progenitors generate trunk neural crest cells in vitro Open
The neural crest (NC) is a multipotent embryonic cell population that generates distinct cell types in an axial position-dependent manner. The production of NC cells from human pluripotent stem cells (hPSCs) is a valuable approach to study…
View article: Author response: Human axial progenitors generate trunk neural crest cells in vitro
Author response: Human axial progenitors generate trunk neural crest cells in vitro Open