Mechanisms of sensorineural cell damage, death and survival in the cochlea

ACY Wong, AF Ryan - Frontiers in aging neuroscience, 2015 - frontiersin.org
The majority of acquired hearing loss, including presbycusis, is caused by irreversible
damage to the sensorineural tissues of the cochlea. This article reviews the intracellular …

Calcium pumps in health and disease

M Brini, E Carafoli - Physiological reviews, 2009 - journals.physiology.org
Ca2+-ATPases (pumps) are key actors in the regulation of Ca2+ in eukaryotic cells and are
thus essential to the correct functioning of the cell machinery. They have high affinity for …

Treatment of monogenic and digenic dominant genetic hearing loss by CRISPR-Cas9 ribonucleoprotein delivery in vivo

Y Tao, V Lamas, W Du, W Zhu, Y Li… - Nature …, 2023 - nature.com
Mutations in Atp2b2, an outer hair cell gene, cause dominant hearing loss in humans. Using
a mouse model Atp2b2 Obl/+, with a dominant hearing loss mutation (Oblivion), we show …

Gene expression by mouse inner ear hair cells during development

DI Scheffer, J Shen, DP Corey… - Journal of …, 2015 - Soc Neuroscience
Hair cells of the inner ear are essential for hearing and balance. As a consequence,
pathogenic variants in genes specifically expressed in hair cells often cause hereditary …

The physiology of mechanoelectrical transduction channels in hearing

R Fettiplace, KX Kim - Physiological reviews, 2014 - journals.physiology.org
Much is known about the mechanotransducer (MT) channels mediating transduction in hair
cells of the vertrbrate inner ear. With the use of isolated preparations, it is experimentally …

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 …

[HTML][HTML] Molecular basis of mechanosensory transduction

PG Gillespie, RG Walker - Nature, 2001 - nature.com
Mechanotransduction—a cell's conversion of a mechanical stimulus into an electrical signal—
reveals vital features of an organism's environment. From hair cells and skin …

[HTML][HTML] The sensory and motor roles of auditory hair cells

R Fettiplace, CM Hackney - Nature reviews neuroscience, 2006 - nature.com
Cochlear hair cells respond with phenomenal speed and sensitivity to sound vibrations that
cause submicron deflections of their hair bundle. Outer hair cells are not only detectors, but …

Structure and innervation of the cochlea

Y Raphael, RA Altschuler - Brain research bulletin, 2003 - Elsevier
The role of the cochlea is to transduce complex sound waves into electrical neural activity in
the auditory nerve. Hair cells of the organ of Corti are the sensory cells of hearing. The inner …

[HTML][HTML] Alterations of the CIB2 calcium-and integrin-binding protein cause Usher syndrome type 1J and nonsyndromic deafness DFNB48

S Riazuddin, IA Belyantseva, APJ Giese, K Lee… - Nature …, 2012 - nature.com
Sensorineural hearing loss is genetically heterogeneous. Here, we report that mutations in
CIB2, which encodes a calcium-and integrin-binding protein, are associated with …