Molecular mechanisms of inner ear development

DK Wu, MW Kelley - Cold Spring Harbor perspectives in …, 2012 - cshperspectives.cshlp.org
The inner ear is a structurally complex vertebrate organ built to encode sound, motion, and
orientation in space. Given its complexity, it is not surprising that inner ear dysfunction is a …

The mechanosensory transduction machinery in inner ear hair cells

W Zheng, JR Holt - Annual review of biophysics, 2021 - annualreviews.org
Sound-induced mechanical stimuli are detected by elaborate mechanosensory transduction
(MT) machinery in highly specialized hair cells of the inner ear. Genetic studies of inherited …

[HTML][HTML] Genetic heterogeneity in human disease

J McClellan, MC King - Cell, 2010 - cell.com
Strong evidence suggests that rare mutations of severe effect are responsible for a
substantial portion of complex human disease. Evolutionary forces generate vast genetic …

[HTML][HTML] Whole exome sequencing and homozygosity mapping identify mutation in the cell polarity protein GPSM2 as the cause of nonsyndromic hearing loss …

T Walsh, H Shahin, T Elkan-Miller, MK Lee… - The American Journal of …, 2010 - cell.com
Massively parallel sequencing of targeted regions, exomes, and complete genomes has
begun to dramatically increase the pace of discovery of genes responsible for human …

Rescue of hearing and vestibular function by antisense oligonucleotides in a mouse model of human deafness

JJ Lentz, FM Jodelka, AJ Hinrich, KE McCaffrey… - Nature medicine, 2013 - nature.com
Hearing impairment is the most common sensory disorder, with congenital hearing
impairment present in approximately 1 in 1,000 newborns. Hereditary deafness is often …

[HTML][HTML] Drosophila auditory organ genes and genetic hearing defects

PR Senthilan, D Piepenbrock, G Ovezmyradov… - Cell, 2012 - cell.com
The Drosophila auditory organ shares equivalent transduction mechanisms with vertebrate
hair cells, and both are specified by atonal family genes. Using a whole-organ knockout …

[HTML][HTML] Mechanical forces drive ordered patterning of hair cells in the mammalian inner ear

R Cohen, L Amir-Zilberstein, M Hersch… - Nature …, 2020 - nature.com
Periodic organization of cells is required for the function of many organs and tissues. The
development of such periodic patterns is typically associated with mechanisms based on …

[HTML][HTML] Targeted genomic capture and massively parallel sequencing to identify genes for hereditary hearing loss in Middle Eastern families

Z Brownstein, LM Friedman, H Shahin, V Oron-Karni… - Genome biology, 2011 - Springer
Background Identification of genes responsible for medically important traits is a major
challenge in human genetics. Due to the genetic heterogeneity of hearing loss, targeted …

[HTML][HTML] Hearing impairment: a panoply of genes and functions

AA Dror, KB Avraham - Neuron, 2010 - cell.com
Research in the genetics of hearing and deafness has evolved rapidly over the past years,
providing the molecular foundation for different aspects of the mechanism of hearing …

Hearing protection, restoration, and regeneration: an overview of emerging therapeutics for inner ear and central hearing disorders

AGM Schilder, MP Su, H Blackshaw, L Lustig… - Otology & …, 2019 - journals.lww.com
Objective: To provide an overview of biotechnology and pharmaceutical companies active in
the field of inner ear and central hearing disorders and their therapeutic approaches …