The RNA-binding protein TRIM71 is essential for hearing in humans and mice and times auditory sensory organ development
Ontology highlight
ABSTRACT:
PROVIDER: PRJNA1183852 | ENA |
REPOSITORIES: ENA
Similar Datasets
Project description:Utilizing conditional Trim71 knockout mice, we find that Trim71 deletion during early otic development causes hearing loss and induces premature cell cycle exit and differentiation of hair cell progenitors. Transcriptomic and genetic experiments reveal that TRIM71 maintains hair cell-progenitors proliferative and undifferentiated through repressing TGF-β-type signaling.
2025-06-21 | GSE281437 | GEO
Project description:As the primary sensory neurons of the auditory system, Type I spiral ganglion neurons (SGNs) encode sound stimulus properties critical for the formation of auditory percept in higher brain areas. Their functional heterogeneity is thought to contribute to our ability to hear a wide range of sound intensities and against background noise, but how SGN diversity arises during development is poorly understood. Here we studied the role of the transcription factor Runx1 in establishing SGN heterogeneity in the mouse cochlea by single cell RNA-sequencing.
2023-01-13 | GSE210216 | GEO
Project description:As the primary sensory neurons of the auditory system, Type I spiral ganglion neurons (SGNs) encode sound stimulus properties critical for the formation of auditory percept in higher brain areas. Their functional heterogeneity is thought to contribute to our ability to hear a wide range of sound intensities and against background noise, but how SGN diversity arises during development is poorly understood. Here we studied the role of the transcription factor Runx1 in establishing SGN heterogeneity in the mouse cochlea by single cell RNA-sequencing.
2023-01-13 | GSE210215 | GEO
Project description:Runx1 Controls Auditory Sensory Neuron Diversity (iCKO)
| PRJNA864782 | ENA
Project description:Runx1 Controls Auditory Sensory Neuron Diversity (CKO)
| PRJNA864783 | ENA
Project description:While low-frequency sounds have been reported to stimulate dispersal responses in male and female mosquitoes, only males show attraction to sound. Male attraction to female flight tones is important during courtship; however, groups of males show diverse responses to acoustic stimuli, suggesting auditory processing can vary drastically between the sexes and individual males. To investigate diversity in auditory representation within and between the sexes, we utilised molecular and functional analyses to explore Aedes aegypti mosquito auditory processing. We identified shared and dimorphic neurons connecting mosquito ears to the brains’ primary auditory processing center. Calcium imaging from this brain region facilitated definition of multiple neuronal clusters based on auditory stimulation responses. More clusters with greater complexity were identified in males than females, with these clusters highly differentiated amongst males. Transcriptomic and proteomic analyses found enrichment of ciliary-related factors in male ears compared to females, potentially underlying sexual dimorphisms in hearing systems.
2025-05-12 | GSE291714 | GEO
Project description:Age-related hearing (ARHL) loss affects a large part of the human population with a major impact on our aging societies. Yet, underlying mechanisms are not understood, and no validated therapy or prevention exists. NADPH oxidases (NOX), are important sources of reactive oxygen species (ROS) in the cochlea and might therefore be involved in the pathogenesis of ARHL. Here we investigate ARHL in a mouse model. Wild type mice showed early loss of hearing and cochlear integrity, while animals deficient in the NOX subunit p22phox remained unaffected up to six months. Genes of the excitatory pathway were down-regulated in p22phox-deficient auditory neurons. Our results demonstrate that NOX activity leads to upregulation of genes of the excitatory pathway, to excitotoxic cochlear damage, and ultimately to ARHL. In the absence of functional NOXs, aging mice conserve hearing and cochlear morphology. Our study offers new insights into pathomechanisms and future therapeutic targets of ARHL.
2020-12-18 | E-MTAB-8668 | biostudies-arrayexpress
Project description:The mammalian auditory sensory organ, the organ of Corti, has no capacity for regenerating lost hair cells, leading to permanent hearing impairment. In this study, we demonstrate that auditory supporting cells resist Yap activation after Hippo inhibition and uncover the molecular mechanism maintaining quiescence in the adult cochlear supporting cells.
2025-04-23 | GSE268504 | GEO
Project description:External Auditory Canal Microbiome of Hearing aid users
| PRJEB65966 | ENA
Project description:Alzheimer's disease (AD) is the most common type of dementia and is characterized by cognitive and behavioral impairment that significantly interferes with social functioning. Over the last decade, there has been an increased interest in whether sensory deficiency may be associated with the development of AD. Notably, the relationship between hearing impairment and AD is of great relevance, but still poorly understood. In this study, we found an early-onset hearing loss in 3xTgAD and 3xTgAD/Polβ+/− mouse models. The hearing assessment was measured by auditory brainstem response (ABR) and distortion product otoacoustic emission (DPOAE) recordings. Herein, we report that both 3xTgAD and 3xTgAD/Polβ+/− mice showed increased ABR thresholds between at 16 and 32 kHz at 4 weeks of age, much earlier than any AD behavioral changes. The ABR thresholds were significantly higher in 3xTgAD/Polβ+/− mice than in 3xTgAD mice at 16 kHz, and DPOAE signals were also reduced, indicating that DNA damage may affect hearing impairment in AD. Poly ADP-ribosylation levels and protein expression of DNA damage markers increased significantly and NAD+ levels were reduced in the cochlea of 3xTgAD and 3xTgAD/Polβ+/− mice. Remarkably, we observed the downregulation of phosphoglycerate mutase 2 (PGAM2) in cochlea and a decrease in the number of synaptic ribbons in the presynaptic zones of inner hair cells in 3xTgAD/Polβ+/− mice. We also find that the activity of sirtuin 3 (SIRT3) is downregulated in cochlea, indicative of impaired mitochondrial function. Taken together, hearing impairment may be a potential early marker of AD. In addition, these findings provide new insights into the mechanism of the relationship between hearing dysfunction and AD and suggest that DNA damage in the cochlea can contributes to the development of early hearing loss of AD. The hearing assessment was measured by auditory brainstem response (ABR) and distortion product otoacoustic emission (DPOAE) at 4 weeks of age, thereafter whole cochlea and auditory cortex tissue were collected from each mouse (WT, Polβ+/−, 3xTgAD, and 3xTgAD/Polβ+/−) and subjected to total RNA isolation to understand what metabolism-related gene expression changes in cochlea and auditory cortex.
2024-03-27 | GSE232205 | GEO