CLC chloride channels and transporters: structure, function, physiology, and disease

TJ Jentsch, M Pusch - Physiological reviews, 2018 - journals.physiology.org
CLC anion transporters are found in all phyla and form a gene family of eight members in
mammals. Two CLC proteins, each of which completely contains an ion translocation …

Hair cell transduction, tuning, and synaptic transmission in the mammalian cochlea

R Fettiplace - Comprehensive Physiology, 2011 - Wiley Online Library
Sound pressure fluctuations striking the ear are conveyed to the cochlea, where they vibrate
the basilar membrane on which sit hair cells, the mechanoreceptors of the inner ear …

Single-cell transcriptomic atlas of mouse cochlear aging

G Sun, Y Zheng, X Fu, W Zhang, J Ren, S Ma… - Protein & …, 2023 - academic.oup.com
Progressive functional deterioration in the cochlea is associated with age-related hearing
loss (ARHL). However, the cellular and molecular basis underlying cochlear aging remains …

The mechanism of action of retigabine (ezogabine), a first‐in‐class K+ channel opener for the treatment of epilepsy

MJ Gunthorpe, CH Large, R Sankar - Epilepsia, 2012 - Wiley Online Library
The pharmacologic profile of retigabine [RTG (international nonproprietary name);
ezogabine, EZG (US adopted name)], is different from all currently approved antiepileptic …

Modulation of Kv7 channels and excitability in the brain

DL Greene, N Hoshi - Cellular and Molecular Life Sciences, 2017 - Springer
Neuronal Kv7 channels underlie a voltage-gated non-inactivating potassium current known
as the M-current. Due to its particular characteristics, Kv7 channels show pronounced …

[HTML][HTML] Prestin-driven cochlear amplification is not limited by the outer hair cell membrane time constant

SL Johnson, M Beurg, W Marcotti, R Fettiplace - Neuron, 2011 - cell.com
Outer hair cells (OHCs) provide amplification in the mammalian cochlea using somatic force
generation underpinned by voltage-dependent conformational changes of the motor protein …

[HTML][HTML] In vivo outer hair cell gene editing ameliorates progressive hearing loss in dominant-negative Kcnq4 murine model

B Noh, JH Rim, R Gopalappa, H Lin, KM Kim… - Theranostics, 2022 - ncbi.nlm.nih.gov
Outer hair cell (OHC) degeneration is a major cause of progressive hearing loss and
presbycusis. Despite the high prevalence of these disorders, targeted therapy is currently …

[HTML][HTML] Precise detection of CRISPR-Cas9 editing in hair cells in the treatment of autosomal dominant hearing loss

C Cui, D Wang, B Huang, F Wang, Y Chen, J Lv… - … Therapy-Nucleic Acids, 2022 - cell.com
Gene therapy would benefit from the effective editing of targeted cells with CRISPR-Cas9
tools. However, it is difficult to precisely assess the editing performance in vivo because the …

Potassium ion movement in the inner ear: insights from genetic disease and mouse models

AA Zdebik, P Wangemann, TJ Jentsch - Physiology, 2009 - journals.physiology.org
Sensory transduction in the cochlea and vestibular labyrinth depends on fluid movements
that deflect the hair bundles of mechanosensitive hair cells. Mechanosensitive transducer …

Hearing loss: mechanisms revealed by genetics and cell biology

AA Dror, KB Avraham - Annual review of genetics, 2009 - annualreviews.org
Hearing loss (HL), or deafness in its most severe form, affects an estimated 28 and 22.5
million Americans and Europeans, respectively. The numbers are higher in regions such as …