Guy P. Richardson
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View article: The <i>TECTB-C225Y</i> Variant Causing Autosomal Dominant Deafness in a Nicaraguan Family Enhances Sensitivity to Noise-Induced Hearing Loss in Mice
The <i>TECTB-C225Y</i> Variant Causing Autosomal Dominant Deafness in a Nicaraguan Family Enhances Sensitivity to Noise-Induced Hearing Loss in Mice Open
Identifying new genes responsible for non-syndromic hearing loss remains a critical goal, as many individuals with hereditary deafness still lack a molecular diagnosis despite comprehensive genetic testing. The tectorial membrane (TM) is a…
View article: Taperin bundles F-actin at stereocilia pivot points enabling optimal lifelong mechanosensitivity
Taperin bundles F-actin at stereocilia pivot points enabling optimal lifelong mechanosensitivity Open
Stereocilia are rod-like mechanosensory projections consisting of unidirectionally oriented actin filaments that extend into the inner ear hair cell cytoskeleton, forming dense rootlets. Taperin (TPRN) localizes to the narrowed-down base o…
View article: Large-scale annotated dataset for cochlear hair cell detection and classification
Large-scale annotated dataset for cochlear hair cell detection and classification Open
Our sense of hearing is mediated by cochlear hair cells, of which there are two types organized in one row of inner hair cells and three rows of outer hair cells. Each cochlea contains 5–15 thousand terminally differentiated hair cells, an…
View article: Large-scale annotated dataset for cochlear hair cell detection and classification
Large-scale annotated dataset for cochlear hair cell detection and classification Open
Our sense of hearing is mediated by cochlear hair cells, of which there are two types organized in one row of inner hair cells and three rows of outer hair cells. Each cochlea contains 5 - 15 thousand terminally differentiated hair cells, …
View article: Large-scale annotated dataset for cochlear hair cell detection and classification
Large-scale annotated dataset for cochlear hair cell detection and classification Open
Our sense of hearing is mediated by cochlear hair cells, of which there are two types organized in one row of inner hair cells and three rows of outer hair cells. Each cochlea contains 5 - 15 thousand terminally differentiated hair cells, …
View article: Large-scale annotated dataset for cochlear hair cell detection and classification
Large-scale annotated dataset for cochlear hair cell detection and classification Open
Our sense of hearing is mediated by cochlear hair cells, of which there are two types organized in one row of inner hair cells and three rows of outer hair cells. Each cochlea contains 5 - 15 thousand terminally differentiated hair cells, …
View article: Large-scale annotated dataset for cochlear hair cell detection and classification
Large-scale annotated dataset for cochlear hair cell detection and classification Open
This is the dataset behind the Hair Cell Analysis Toolbox (HCAT)
View article: Charge and lipophilicity are required for effective block of the hair-cell mechano-electrical transducer channel by FM1-43 and its derivatives
Charge and lipophilicity are required for effective block of the hair-cell mechano-electrical transducer channel by FM1-43 and its derivatives Open
The styryl dye FM1-43 is widely used to study endocytosis but behaves as a permeant blocker of the mechano-electrical transducer (MET) channel in sensory hair cells, loading rapidly and specifically into the cytoplasm of hair cells in a ME…
View article: Large-scale annotated dataset for cochlear hair cell detection and classification
Large-scale annotated dataset for cochlear hair cell detection and classification Open
Our sense of hearing is mediated by cochlear hair cells, localized within the sensory epithelium called the organ of Corti. There are two types of hair cells in the cochlea, which are organized in one row of inner hair cells and three rows…
View article: Large-scale annotated dataset for cochlear hair cell detection and classification
Large-scale annotated dataset for cochlear hair cell detection and classification Open
This is the dataset behind the Hair Cell Analysis Toolbox (HCAT)
View article: AAV-mediated rescue of Eps8 expression in vivo restores hair-cell function in a mouse model of recessive deafness
AAV-mediated rescue of Eps8 expression in vivo restores hair-cell function in a mouse model of recessive deafness Open
The transduction of acoustic information by hair cells depends upon mechanosensitive stereociliary bundles that project from their apical surface. Mutations or absence of the stereociliary protein EPS8 cause deafness in humans and mice, re…
View article: Effect of boundary conditions on a high-performance isolation hexapod platform
Effect of boundary conditions on a high-performance isolation hexapod platform Open
Isolation of spacecraft microvibrations is essential for the successful deployment of instruments relying on high-precision pointing. Hexapod platforms represent a promising solution, but the difficulties associated with attaining desirabl…
View article: Identification of a series of hair-cell MET channel blockers that protect against aminoglycoside-induced ototoxicity
Identification of a series of hair-cell MET channel blockers that protect against aminoglycoside-induced ototoxicity Open
To identify small molecules that shield mammalian sensory hair cells from the ototoxic side effects of aminoglycoside antibiotics, 10,240 compounds were initially screened in zebrafish larvae, selecting for those that protected lateral-lin…
View article: MET currents and otoacoustic emissions from mice with a detached tectorial membrane indicate the extracellular matrix regulates Ca<sup>2+</sup> near stereocilia
MET currents and otoacoustic emissions from mice with a detached tectorial membrane indicate the extracellular matrix regulates Ca<sup>2+</sup> near stereocilia Open
Key points The aim was to determine whether detachment of the tectorial membrane (TM) from the organ of Corti in Tecta/Tectb −/− mice affects the biophysical properties of cochlear outer hair cells (OHCs). Tecta/Tectb −/− mice have highly …
View article: Age‐related changes in the biophysical and morphological characteristics of mouse cochlear outer hair cells
Age‐related changes in the biophysical and morphological characteristics of mouse cochlear outer hair cells Open
Key points Age‐related hearing loss (ARHL) is a very heterogeneous disease, resulting from cellular senescence, genetic predisposition and environmental factors (e.g. noise exposure). Currently, we know very little about age‐related change…
View article: Gentamicin Affects the Bioenergetics of Isolated Mitochondria and Collapses the Mitochondrial Membrane Potential in Cochlear Sensory Hair Cells
Gentamicin Affects the Bioenergetics of Isolated Mitochondria and Collapses the Mitochondrial Membrane Potential in Cochlear Sensory Hair Cells Open
Aminoglycoside antibiotics are widely prescribed to treat a variety of serious bacterial infections. They are extremely useful clinical tools, but have adverse side effects such as oto- and nephrotoxicity. Once inside a cell they are thoug…
View article: ORC-13661 protects sensory hair cells from aminoglycoside and cisplatin ototoxicity
ORC-13661 protects sensory hair cells from aminoglycoside and cisplatin ototoxicity Open
Aminoglycoside (AG) antibiotics are widely used to prevent life-threatening infections, and cisplatin is used in the treatment of various cancers, but both are ototoxic and result in loss of sensory hair cells from the inner ear. ORC-13661…
View article: Accelerated Age-Related Degradation of the Tectorial Membrane in the Ceacam16βgal/βgal Null Mutant Mouse, a Model for Late-Onset Human Hereditary Deafness DFNB113
Accelerated Age-Related Degradation of the Tectorial Membrane in the Ceacam16βgal/βgal Null Mutant Mouse, a Model for Late-Onset Human Hereditary Deafness DFNB113 Open
CEACAM16 is a non-collagenous protein of the tectorial membrane, an extracellular structure of the cochlea essential for normal hearing. Dominant and recessive mutations in CEACAM16 have been reported to cause postlingual and progressive f…
View article: Design, Synthesis, and Biological Evaluation of a New Series of Carvedilol Derivatives That Protect Sensory Hair Cells from Aminoglycoside-Induced Damage by Blocking the Mechanoelectrical Transducer Channel
Design, Synthesis, and Biological Evaluation of a New Series of Carvedilol Derivatives That Protect Sensory Hair Cells from Aminoglycoside-Induced Damage by Blocking the Mechanoelectrical Transducer Channel Open
Aminoglycosides (AGs) are broad-spectrum antibiotics used for the treatment of serious bacterial infections but have use-limiting side effects including irreversible hearing loss. Here, we assessed the otoprotective profile of carvedilol i…
View article: Hair-Bundle Links: Genetics as the Gateway to Function
Hair-Bundle Links: Genetics as the Gateway to Function Open
Up to five distinct cell-surface specializations interconnect the stereocilia and the kinocilium of the mature hair bundle in some species: kinocilial links, tip links, top connectors, shaft connectors, and ankle links. In developing hair …
View article: Spontaneous Otoacoustic Emissions in<i>Tecta<sup>Y1870C/+</sup></i>Mice Reflect Changes in Cochlear Amplification and How It Is Controlled by the Tectorial Membrane
Spontaneous Otoacoustic Emissions in<i>Tecta<sup>Y1870C/+</sup></i>Mice Reflect Changes in Cochlear Amplification and How It Is Controlled by the Tectorial Membrane Open
Spontaneous otoacoustic emissions (SOAEs) recorded from the ear canal in the absence of sound reflect cochlear amplification, an outer hair cell (OHC) process required for the extraordinary sensitivity and frequency selectivity of mammalia…
View article: FGFR1-mediated protocadherin-15 loading mediates cargo specificity during intraflagellar transport in inner ear hair-cell kinocilia
FGFR1-mediated protocadherin-15 loading mediates cargo specificity during intraflagellar transport in inner ear hair-cell kinocilia Open
Significance Hair cells, the inner ear mechanosensory receptors, are responsible for hearing and balance. This occurs through a specialized apical organelle known as the hair bundle, which is made up of actin-based projections called stere…
View article: TectaY1870C/+ mice with alterations in the structure and porosity of the tectorial membrane display large numbers of spontaneous emissions
TectaY1870C/+ mice with alterations in the structure and porosity of the tectorial membrane display large numbers of spontaneous emissions Open
Spontaneous otoacoustic emissions (SOAEs) are a signature of cochlear amplification, a process associated with outer hair cell (OHC) function and required for the sensitivity and frequency selectivity of cochlear responses. Although normal…
View article: Identification of ion-channel modulators that protect against aminoglycoside-induced hair cell death
Identification of ion-channel modulators that protect against aminoglycoside-induced hair cell death Open
Aminoglycoside antibiotics are used to treat life-threatening bacterial infections but can cause deafness due to hair cell death in the inner ear. Compounds have been described that protect zebrafish lateral line hair cells from aminoglyco…
View article: TMC2 Modifies Permeation Properties of the Mechanoelectrical Transducer Channel in Early Postnatal Mouse Cochlear Outer Hair Cells
TMC2 Modifies Permeation Properties of the Mechanoelectrical Transducer Channel in Early Postnatal Mouse Cochlear Outer Hair Cells Open
The ability of cochlear hair cells to convert sound into receptor potentials relies on the mechanoelectrical transducer (MET) channels present in their stereociliary bundles. There is strong evidence implying that transmembrane channel-lik…