Rescue of autosomal dominant hearing loss by in vivo delivery of mini dCas13X-derived RNA base editor

Q Xiao, Z Xu, Y Xue, C Xu, L Han, Y Liu… - Science Translational …, 2022 - science.org
Programmable RNA editing tools enable the reversible correction of mutant transcripts,
reducing the potential risk associated with permanent genetic changes associated with the …

Synaptic transmission at the vestibular hair cells of amniotes

M Mukhopadhyay, T Pangrsic - Molecular and Cellular Neuroscience, 2022 - Elsevier
A harmonized interplay between the central nervous system and the five peripheral end
organs is how the vestibular system helps organisms feel a sense of balance and motion in …

[HTML][HTML] Animal-to-human translation difficulties and problems with proposed coding-in-noise deficits in noise-induced synaptopathy and hidden hearing loss

S Ripley, L Xia, Z Zhang, SJ Aiken… - Frontiers in Neuroscience, 2022 - frontiersin.org
Noise induced synaptopathy (NIS) and hidden hearing loss (NIHHL) have been hot topic in
hearing research since a massive synaptic loss was identified in CBA mice after a brief …

[HTML][HTML] Gene editing in a Myo6 semi-dominant mouse model rescues auditory function

Y Xue, X Hu, D Wang, D Li, Y Li, F Wang, M Huang… - Molecular Therapy, 2022 - cell.com
Myosin VI (MYO6) is an unconventional myosin that is vital for auditory and vestibular
function. Pathogenic variants in the human MYO6 gene cause autosomal-dominant or …

[HTML][HTML] Local magnetic delivery of adeno-associated virus AAV2 (quad YF)-mediated BDNF gene therapy restores hearing after noise injury

S Mukherjee, M Kuroiwa, W Oakden, BT Paul… - Molecular Therapy, 2022 - cell.com
Moderate noise exposure may cause acute loss of cochlear synapses without affecting the
cochlear hair cells and hearing threshold; thus, it remains" hidden" to standard clinical tests …

[HTML][HTML] PTEN inhibitor bisperoxovanadium protects against noise-induced hearing loss

B Fan, F Lu, WJ Du, J Chen, XG An… - Neural Regeneration …, 2023 - journals.lww.com
Studies have shown that phosphatase and tensin homolog deleted on chromosome ten
(PTEN) participates in the regulation of cochlear hair cell survival. Bisperoxovanadium …

[HTML][HTML] Disruption of Hars2 in Cochlear Hair Cells Causes Progressive Mitochondrial Dysfunction and Hearing Loss in Mice

P Xu, L Wang, H Peng, H Liu, H Liu, Q Yuan… - Frontiers in Cellular …, 2021 - frontiersin.org
Mutations in a number of genes encoding mitochondrial aminoacyl-tRNA synthetases lead
to non-syndromic and/or syndromic sensorineural hearing loss in humans, while their …

[HTML][HTML] Changes in microRNA expression in the cochlear nucleus and inferior colliculus after acute noise-induced hearing loss

S Park, SH Han, BG Kim, MW Suh, JH Lee… - International Journal of …, 2020 - mdpi.com
Noise-induced hearing loss (NIHL) can lead to secondary changes that induce neural
plasticity in the central auditory pathway. These changes include decreases in the number of …

Protection of cochlear ribbon synapses and prevention of hidden hearing loss

M Wei, W Wang, Y Liu, X Mao, TS Chen… - Neural Plasticity, 2020 - Wiley Online Library
In the auditory system, ribbon synapses are vesicle‐associated structures located between
inner hair cells (IHCs) and spiral ganglion neurons that are implicated in the modulation of …

[HTML][HTML] Noise exposure potentiates exocytosis from cochlear inner hair cells

LE Boero, S Payne, ME Gómez-Casati… - Frontiers in synaptic …, 2021 - frontiersin.org
Noise-induced hearing loss has gained relevance as one of the most common forms of
hearing impairment. The anatomical correlates of hearing loss, principally cell damage …