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View article: Rare Duplication in the RYR1 Gene Causing Malignant Hyperthermia and Clinical Variability
Rare Duplication in the RYR1 Gene Causing Malignant Hyperthermia and Clinical Variability Open
Background/Objectives: Variants in the RYR1 gene are associated mainly with Malignant Hyperthermia. Missense variants are largely the most common, while insertions and duplications account for less than 10%. We aimed to investigate the eff…
View article: <scp>mTORC2</scp> Regulates Actin Polymerization in Auditory Cells
<span>mTORC2</span> Regulates Actin Polymerization in Auditory Cells Open
Mammalian target of rapamycin complex 2 (mTORC2) is essential for hearing by regulating auditory hair cell structure and function. However, mechanistic details of how mTORC2 regulates intracellular processes in sensory hair cells have not …
View article: mTORC2 regulates auditory hair cell structure and function
mTORC2 regulates auditory hair cell structure and function Open
mTOR broadly controls cell growth, but little is known about the role of mTOR complex 2 (mTORC2) in the inner ear. To investigate the role of mTORC2 in sensory hair cells (HCs), we generated HC-specific Rictor knockout (HC-RicKO) mice. HC-…
View article: Exogenous humanin and MOTS-c function as protective agents against gentamicin-induced hair cell damage
Exogenous humanin and MOTS-c function as protective agents against gentamicin-induced hair cell damage Open
Loss of hair cells can lead to irreversible sensorineural hearing loss. Therefore, hair cell preservation is critical for hearing. Mitochondrial derived peptides (MDPs) are bioactive peptides and prominent members of this family are humani…
View article: Zebrafish (Danio rerio) larvae as a predictive model to study gentamicin-induced structural alterations of the kidney
Zebrafish (Danio rerio) larvae as a predictive model to study gentamicin-induced structural alterations of the kidney Open
Nephrotoxicity is an important drug safety aspect to be assessed during drug discovery and development. To study renal toxicity, in vitro cell-based assays are often used. Unfortunately, translating the results of such cell assays to verte…
View article: Zebrafish (Danio rerio) larvae as a predictive model to study gentamicin-induced structural alterations of renal organs
Zebrafish (Danio rerio) larvae as a predictive model to study gentamicin-induced structural alterations of renal organs Open
This repository contains reconstructed µCT scans acquired at the SOLEIL synchrotron facility which were analyzed in the above mentioned publication. The first set of scans shows mouse kidney biopsies. One representative scan from the contr…
View article: A deep learning approach to quantify auditory hair cells
A deep learning approach to quantify auditory hair cells Open
Hearing loss affects millions of people worldwide. Yet, there are still no curative therapies for sensorineural hearing loss. Frequent causes of sensorineural hearing loss are due to damage or loss of the sensory hair cells, the spiral gan…
View article: mTOR Signaling in the Inner Ear as Potential Target to Treat Hearing Loss
mTOR Signaling in the Inner Ear as Potential Target to Treat Hearing Loss Open
Hearing loss affects many people worldwide and occurs often as a result of age, ototoxic drugs and/or excessive noise exposure. With a growing number of elderly people, the number of people suffering from hearing loss will also increase in…
View article: A deep learning approach to quantify auditory hair cells
A deep learning approach to quantify auditory hair cells Open
StarDist 2D deep learning model and training dataset.
View article: STAT1 deficiency predisposes to spontaneous otitis media
STAT1 deficiency predisposes to spontaneous otitis media Open
Signal transducer and activator of transcription 1 (STAT1) is known to be an important player in inflammatory responses. STAT1 as a transcription factor regulates the expression of multiple proinflammatory genes. Inflammatory response is o…
View article: Telmisartan Protects Auditory Hair Cells from Gentamicin-Induced Toxicity in vitro
Telmisartan Protects Auditory Hair Cells from Gentamicin-Induced Toxicity in vitro Open
Background: Telmisartan is an angiotensin II receptor blocker that has pleiotropic effects and protective properties in different cell types. Moreover, telmisartan has also shown partial agonism on the peroxisome proliferator-activated rec…
View article: Inner ear exosomes and their potential use as biomarkers
Inner ear exosomes and their potential use as biomarkers Open
Exosomes are nanovesicles involved in intercellular communications. They are released by a variety of cell types; however, their presence in the inner ear has not been described in the literature. The aims of this study were to determine i…
View article: Induction of mitophagy in the HEI-OC1 auditory cell line and activation of the Atg12/LC3 pathway in the organ of Corti
Induction of mitophagy in the HEI-OC1 auditory cell line and activation of the Atg12/LC3 pathway in the organ of Corti Open
Mitochondrial dysfunction has been reported in several neurodegenerative disorders. Since the possible involvement of mitochondria in acquired hearing loss remains uncertain, we focused on the study of mitophagy, a selective intracellular …
View article: Effects of peroxisome proliferator activated receptors (PPAR)-γ and -α agonists on cochlear protection from oxidative stress
Effects of peroxisome proliferator activated receptors (PPAR)-γ and -α agonists on cochlear protection from oxidative stress Open
Various insults cause ototoxicity in mammals by increasing oxidative stress leading to apoptosis of auditory hair cells (HCs). The thiazolidinediones (TZDs; e.g., pioglitazone) and fibrate (e.g., fenofibrate) drugs are used for the treatme…
View article: Sesn2/AMPK/mTOR signaling mediates balance between survival and apoptosis in sensory hair cells under stress
Sesn2/AMPK/mTOR signaling mediates balance between survival and apoptosis in sensory hair cells under stress Open
Sensory hair cells located in the inner ear are essential for detecting sounds. Hair cells can be damaged by noise exposure, aminoglycoside antibiotics, chemotherapeutic agents and effects of aging. In mammals, hair cells do not regenerate…
View article: Brimonidine Protects Auditory Hair Cells from in vitro-Induced Toxicity of Gentamicin
Brimonidine Protects Auditory Hair Cells from in vitro-Induced Toxicity of Gentamicin Open
Brimonidine, an alpha-2 adrenergic receptor (α2-AR) agonist, has neuroprotective effects in the visual system and in spiral ganglion neurons. Auditory hair cells (HCs) express all 3 α2-AR subtypes, but their roles in HCs remain unknown. Th…