Structure, function, and pharmacology of glutamate receptor ion channels

KB Hansen, LP Wollmuth, D Bowie, H Furukawa… - Pharmacological …, 2021 - Elsevier
Many physiologic effects of l-glutamate, the major excitatory neurotransmitter in the
mammalian central nervous system, are mediated via signaling by ionotropic glutamate …

Gene therapy for hearing loss

R Omichi, SB Shibata, CC Morton… - Human molecular …, 2019 - academic.oup.com
Sensorineural hearing loss (SNHL) is the most common sensory disorder. Its underlying
etiologies include a broad spectrum of genetic and environmental factors that can lead to …

[HTML][HTML] Sensory neuron diversity in the inner ear is shaped by activity

BR Shrestha, C Chia, L Wu, SG Kujawa, MC Liberman… - Cell, 2018 - cell.com
In the auditory system, type I spiral ganglion neurons (SGNs) convey complex acoustic
information from inner hair cells (IHCs) to the brainstem. Although SGNs exhibit variation in …

Hair cell mechanotransduction regulates spontaneous activity and spiral ganglion subtype specification in the auditory system

S Sun, T Babola, G Pregernig, KS So, M Nguyen… - Cell, 2018 - cell.com
Type I spiral ganglion neurons (SGNs) transmit sound information from cochlear hair cells to
the CNS. Using transcriptome analysis of thousands of single neurons, we demonstrate that …

A dual‐AAV approach restores fast exocytosis and partially rescues auditory function in deaf otoferlin knock‐out mice

H Al‐Moyed, AP Cepeda, SY Jung, T Moser… - EMBO molecular …, 2019 - embopress.org
Normal hearing and synaptic transmission at afferent auditory inner hair cell (IHC) synapses
require otoferlin. Deafness DFNB 9, caused by mutations in the OTOF gene encoding …

Lgr5+ cells regenerate hair cells via proliferation and direct transdifferentiation in damaged neonatal mouse utricle

T Wang, R Chai, GS Kim, N Pham, L Jansson… - Nature …, 2015 - nature.com
Recruitment of endogenous progenitors is critical during tissue repair. The inner ear utricle
requires mechanosensory hair cells (HCs) to detect linear acceleration. After damage, non …

Developmental refinement of hair cell synapses tightens the coupling of Ca2+ influx to exocytosis

AB Wong, MA Rutherford, M Gabrielaitis… - The EMBO …, 2014 - embopress.org
Cochlear inner hair cells (IHC s) develop from pre‐sensory pacemaker to sound transducer.
Here, we report that this involves changes in structure and function of the ribbon synapses …

Anatomical and physiological development of the human inner ear

R Lim, AM Brichta - Hearing research, 2016 - Elsevier
We describe the development of the human inner ear with the invagination of the otic vesicle
at 4 weeks gestation (WG), the growth of the semicircular canals from 5 WG, and the …

Map** developmental maturation of inner hair cell ribbon synapses in the apical mouse cochlea

S Michanski, K Smaluch, AM Steyer… - Proceedings of the …, 2019 - National Acad Sciences
Ribbon synapses of cochlear inner hair cells (IHCs) undergo molecular assembly and
extensive functional and structural maturation before hearing onset. Here, we characterized …

The synaptic ribbon is critical for sound encoding at high rates and with temporal precision

P Jean, D Lopez de la Morena, S Michanski… - Elife, 2018 - elifesciences.org
We studied the role of the synaptic ribbon for sound encoding at the synapses between
inner hair cells (IHCs) and spiral ganglion neurons (SGNs) in mice lacking RIBEYE …