keyword
https://read.qxmd.com/read/38498576/altered-fhod3-expression-involved-in-progressive-high-frequency-hearing-loss-via-dysregulation-of-actin-polymerization-stoichiometry-in-the-cuticular-plate
#1
JOURNAL ARTICLE
Ely Cheikh Boussaty, Yuzuru Ninoyu, Leonardo R Andrade, Qingzhong Li, Ryu Takeya, Hideki Sumimoto, Takahiro Ohyama, Karl J Wahlin, Uri Manor, Rick A Friedman
Age-related hearing loss (ARHL) is a common sensory impairment with complex underlying mechanisms. In our previous study, we performed a meta-analysis of genome-wide association studies (GWAS) in mice and identified a novel locus on chromosome 18 associated with ARHL specifically linked to a 32 kHz tone burst stimulus. Consequently, we investigated the role of Formin Homology 2 Domain Containing 3 (Fhod3), a newly discovered candidate gene for ARHL based on the GWAS results. We observed Fhod3 expression in auditory hair cells (HCs) primarily localized at the cuticular plate (CP)...
March 18, 2024: PLoS Genetics
https://read.qxmd.com/read/38497652/research-application-of-laser-induced-shock-wave-for-studying-blast-induced-cochlear-injury
#2
JOURNAL ARTICLE
Takaomi Kurioka, Kunio Mizutari, Katsuki Niwa, Eiko Kimura, Satoko Kawauchi, Yasushi Kobayashi, Shunichi Sato
The ear is the organ most susceptible to explosion overpressure, and cochlear injuries frequently occur after blast exposure. Blast exposure can lead to sensorineural hearing loss (SNHL), which is an irreversible hearing loss that negatively affects the quality of life. Detailed blast-induced cochlear pathologies, such as the loss of hair cells, spiral ganglion neurons, cochlear synapses, and disruption of stereocilia, have been previously documented. However, determining cochlear sensorineural deterioration after a blast injury is challenging because animals exposed to blast overpressure usually experience tympanic membrane perforation (TMP), which causes concurrent conductive hearing loss...
March 1, 2024: Journal of Visualized Experiments: JoVE
https://read.qxmd.com/read/38496629/pkhd1l1-is-required-for-stereocilia-bundle-maintenance-prolonged-hearing-function-and-enhanced-resilience-to-noise-exposure
#3
Olga S Strelkova, Richard T Osgood, Chunjie J Tian, Xinyuan Zhang, Evan Hale, Pedro De-la-Torre, Daniel M Hathaway, Artur A Indzhykulian
Sensory hair cells of the cochlea are essential for hearing, relying on the mechanosensitive stereocilia bundle at their apical pole for their function. Polycystic Kidney and Hepatic Disease 1-Like 1 (PKHD1L1) is a stereocilia protein required for normal hearing in mice, and for the formation of the transient stereocilia surface coat, expressed during early postnatal development. While the function of the stereocilia coat remains unclear, growing evidence supports PKHD1L1 as a human deafness gene. In this study we carry out in depth characterization of PKHD1L1 expression in mice during development and adulthood, analyze hair-cell bundle morphology and hearing function in aging PKHD1L1-defficient mouse lines, and assess their susceptibility to noise damage...
March 4, 2024: bioRxiv
https://read.qxmd.com/read/38470372/the-impact-of-the-fluid-solid-coupling-behavior-of-macro-and-micro-structures-in-the-spiral-cochlea-on-hearing
#4
JOURNAL ARTICLE
Zhengshan Zhao, Junyi Liang, Wenjuan Yao
The cilia of the outer hair cells (OHCs) are the key micro-structures involved in cochlear acoustic function, and their interactions with lymph in the cochlea involve complex, highly nonlinear, coupled motion and energy conversions, including macroscopic fluid?solid coupling. Recent optical measurements have shown that the frequency selectivity of the cochlea at high sound levels is entirely mechanical and is determined by the interactions of the hair bundles with the surrounding fluid. In this paper, an analytical mathematical model of the spiral cochlea containing macro- and micro-measurements was developed to investigate how the phonosensitive function of OHCs' motions is influenced by the macro- structural and micro-structural fluid?solid coupling in the spiral cochlea...
March 12, 2024: Journal of Biomechanical Engineering
https://read.qxmd.com/read/38459354/pkhd1l1-a-gene-involved-in-the-stereocilia-coat-causes-autosomal-recessive-nonsyndromic-hearing-loss
#5
JOURNAL ARTICLE
Shelby E Redfield, Pedro De-la-Torre, Mina Zamani, Hanjun Wang, Hina Khan, Tyler Morris, Gholamreza Shariati, Majid Karimi, Margaret A Kenna, Go Hun Seo, Hongen Xu, Wei Lu, Sadaf Naz, Hamid Galehdari, Artur A Indzhykulian, A Eliot Shearer, Barbara Vona
Identification of genes associated with nonsyndromic hearing loss is a crucial endeavor given the substantial number of individuals who remain without a diagnosis after even the most advanced genetic testing. PKHD1L1 was established as necessary for the formation of the cochlear hair-cell stereociliary coat and causes hearing loss in mice and zebrafish when mutated. We sought to determine if biallelic variants in PKHD1L1 also cause hearing loss in humans. Exome sequencing was performed on DNA of four families segregating autosomal recessive nonsyndromic sensorineural hearing loss...
March 9, 2024: Human Genetics
https://read.qxmd.com/read/38440220/ototoxicity-of-polystyrene-nanoplastics-in-mice-hei-oc1-cells-and-zebrafish
#6
JOURNAL ARTICLE
Yuancheng Wu, Lianzhen Li, Lihuan Tang, Willie Peijnenburg, Huangruici Zhang, Daoli Xie, Ruishuang Geng, Tihua Zheng, Liyan Bi, Xiaodan Wei, Han-Jung Chae, Lan Wang, Li Zhao, Bo Li, Qingyin Zheng
Polystyrene nanoplastics are a novel class of pollutants. They are easily absorbed by living organisms, and their potential toxicity has raised concerns. However, the impact of polystyrene nanoplastics on auditory organs remains unknown. Here, our results showed that polystyrene nanoplastics entered the cochlea of mice, HEI-OC1 cells, and lateral line hair cells of zebrafish, causing cellular injury and increasing apoptosis. Additionally, we found that exposure to polystyrene nanoplastics resulted in a significant elevation in the auditory brainstem response thresholds, a loss of auditory sensory hair cells, stereocilia degeneration and a decrease in expression of Claudin-5 and Occludin proteins at the blood-lymphatic barrier in mice...
2024: Frontiers in Molecular Neuroscience
https://read.qxmd.com/read/38408254/disruption-of-cdh23-exon-68-splicing-leads-to-progressive-hearing-loss-in-mice-by-affecting-tip-link-stability
#7
JOURNAL ARTICLE
Nana Li, Shuang Liu, Dange Zhao, Haibo Du, Yuehui Xi, Xiaoxi Wei, Qingling Liu, Ulrich Müller, Qing Lu, Wei Xiong, Zhigang Xu
Inner ear hair cells are characterized by the F-actin-based stereocilia that are arranged into a staircase-like pattern on the apical surface of each hair cell. The tips of shorter-row stereocilia are connected with the shafts of their neighboring taller-row stereocilia through extracellular links named tip links, which gate mechano-electrical transduction (MET) channels in hair cells. Cadherin 23 (CDH23) forms the upper part of tip links, and its cytoplasmic tail is inserted into the so-called upper tip-link density (UTLD) that contains other proteins such as harmonin...
March 5, 2024: Proceedings of the National Academy of Sciences of the United States of America
https://read.qxmd.com/read/38376199/keratan-sulfate-an-electrosensory-neurosentient-bioresponsive-cell-instructive-glycosaminoglycan
#8
JOURNAL ARTICLE
James Melrose
The roles of keratan sulfate (KS) as a proton detection glycosaminoglycan in neurosensory processes in the central and peripheral nervous systems is reviewed. The functional properties of the KS-proteoglycans aggrecan, phosphacan, podocalyxcin as components of perineuronal nets in neurosensory processes in neuronal plasticity, cognitive learning and memory are also discussed. KS-glycoconjugate neurosensory gels used in electrolocation in elasmobranch fish species and KS substituted mucin like conjugates in some tissue contexts in mammals need to be considered in sensory signalling...
February 20, 2024: Glycobiology
https://read.qxmd.com/read/38374196/inhibition-of-the-ilk-akt-pathway-by-upregulation-of-parvb-contributes-to-the-cochlear-cell-death-in-fascin2-gene-knockout-mice
#9
JOURNAL ARTICLE
Rongrong Liu, Wenjing Shang, Yingying Liu, Yi Xie, Jun Luan, Ting Zhang, Ying Ma, Zengxian Wang, Yan Sun, Xicheng Song, Fengchan Han
The Fscn2 (Fascin2) gene encodes an actin cross-linking protein that is involved in the formation of hair cell stereocilia and retina structure. Mutations in Fscn2 gene have been linked to hearing impairment and retinal degeneration in humans and mice. To understand the function of the Fscn2 gene, we generated the Fscn2 knockout mice, which showed progressive loss of hearing and hair cells. Our goal of the present study was to investigate the mechanism underlying cochlear cell death in the Fscn2 knockout mice...
February 19, 2024: Cell Death Discovery
https://read.qxmd.com/read/38343799/sub-immunogold-sem-reveals-nanoscale-distribution-of-submembranous-epitopes
#10
Katharine K Miller, Pei Wang, Nicolas Grillet
Electron microscopy paired with immunogold labeling is the most precise tool for protein localization. However, these methods are either cumbersome, resulting in small sample numbers and restricted quantification, or limited to identifying protein epitopes external to the membrane. Here, we introduce SUB-immunogold-SEM, a scanning electron microscopy technique that detects intracellular protein epitopes proximal to the membrane. We identified four critical sample preparation factors that contribute to the method's sensitivity and validate its efficacy through precise localization and high-powered quantification of cytoskeletal and transmembrane proteins...
January 22, 2024: Research Square
https://read.qxmd.com/read/38338845/cellular-distribution-of-aquaporin-3-7-and-9-in-the-male-reproductive-system-a-lesson-from-bovine-study-bos-taurus
#11
JOURNAL ARTICLE
Patrycja Oberska, Marta Grabowska, Marta Marynowska, Maciej Murawski, Dariusz Gączarzewicz, Andrzej Syczewski, Katarzyna Michałek
The increasing incidence of male infertility in humans and animals creates the need to search for new factors that significantly affect the course of reproductive processes. Therefore, the aim of this study was to determine the temporospatial expression of aquaglyceroporins (AQP3, AQP7 and AQP9) in the bovine ( Bos taurus ) reproductive system using immunohistochemistry and Western blotting. The study also included morphological analysis and identification of GATA-4. In brief, in immature individuals, AQP3 and AQP7 were found in gonocytes...
January 26, 2024: International Journal of Molecular Sciences
https://read.qxmd.com/read/38277880/regional-differences-in-cochlear-nonlinearity-across-the-basal-organ-of-corti-of-gerbil-regional-differences-in-cochlear-nonlinearity
#12
JOURNAL ARTICLE
C Elliott Strimbu, Lauren A Chiriboga, Brian L Frost, Elizabeth S Olson
Auditory sensation is based in nanoscale vibration of the sensory tissue of the cochlea, the organ of Corti complex (OCC). Motion within the OCC is now observable due to optical coherence tomography. In a previous study (Cooper et al., 2018), the region that includes the electro-motile outer hair cells (OHC) and Deiters cells (DC) was observed to move with larger amplitude than the basilar membrane (BM) and surrounding regions and was termed the "hotspot." In addition to this quantitative distinction, the hotspot moved qualitatively differently than the BM, in that its motion scaled nonlinearly with stimulus level at all frequencies, evincing sub-BF activity...
January 12, 2024: Hearing Research
https://read.qxmd.com/read/38260480/loxhd1-is-indispensable-for-coupling-auditory-mechanosensitive-channels-to-the-site-of-force-transmission
#13
Nicolas Grillet, Pei Wang, Katharine Miller, Enqi He, Siddhant Dhawan, Christopher Cunningham
Hearing is initiated in hair cells by the mechanical activation of ion channels in the hair bundle. The hair bundle is formed by stereocilia organized into rows of increasing heights interconnected by tip links, which convey sound-induced forces to stereocilia tips. The auditory mechanosensitive channels are complexes containing at least four protein-subunits - TMC1/2, TMIE, CIB2, and LHFPL5 - and are located at the tips of shorter stereocilia at a yet-undetermined distance from the lower tip link insertion point...
January 2, 2024: Research Square
https://read.qxmd.com/read/38260322/fascin-structural-plasticity-mediates-flexible-actin-bundle-construction
#14
Rui Gong, Matthew J Reynolds, Keith Hamilton, Gregory M Alushin
Fascin crosslinks actin filaments (F-actin) into bundles that support tubular membrane protrusions including filopodia, stereocilia, and microvilli. Fascin dysregulation drives aberrant cell migration during metastasis, and fascin inhibitors are under development as cancer therapeutics. Here, we use cryo-electron microscopy and cryo-electron tomography coupled with custom denoising to probe fascin's F-actin crosslinking mechanisms across spatial scales. Our fascin crossbridge structure reveals an asymmetric F-actin binding conformation that is allosterically blocked by the inhibitor G2...
January 4, 2024: bioRxiv
https://read.qxmd.com/read/38243601/clarin-2-gene-supplementation-durably-preserves-hearing-in-a-model-of-progressive-hearing-loss
#15
JOURNAL ARTICLE
Clara Mendia, Thibault Peineau, Mina Zamani, Chloé Felgerolle, Nawal Yahiaoui, Nele Christophersen, Samantha Papal, Audrey Maudoux, Reza Maroofian, Pranav Patni, Sylvie Nouaille, Michael R Bowl, Sedigheh Delmaghani, Hamid Galehdari, Barbara Vona, Didier Dulon, Sandrine Vitry, Aziz El-Amraoui
Hearing loss is a major health concern affecting millions of people worldwide with currently limited treatment options. In clarin-2 deficient Clrn2-/- mice, used here as a model of progressive hearing loss, we report synaptic auditory abnormalities in addition to the previously demonstrated defects of hair bundle structure and mechanoelectrical transduction. We sought an in-depth evaluation of viral-mediated gene delivery as a therapy for these hearing-impaired mice. Supplementation with either the murine Clrn2 or human CLRN2 genes preserved normal hearing in treated Clrn2-/- mice...
January 18, 2024: Molecular Therapy
https://read.qxmd.com/read/38228630/the-piezo-channel-is-a-mechano-sensitive-complex-component-in-the-mammalian-inner-ear-hair-cell
#16
JOURNAL ARTICLE
Jeong Han Lee, Maria C Perez-Flores, Seojin Park, Hyo Jeong Kim, Yingying Chen, Mincheol Kang, Jennifer Kersigo, Jinsil Choi, Phung N Thai, Ryan L Woltz, Dolores Columba Perez-Flores, Guy Perkins, Choong-Ryoul Sihn, Pauline Trinh, Xiao-Dong Zhang, Padmini Sirish, Yao Dong, Wayne Wei Feng, Isaac N Pessah, Rose E Dixon, Bernd Sokolowski, Bernd Fritzsch, Nipavan Chiamvimonvat, Ebenezer N Yamoah
The inner ear is the hub where hair cells (HCs) transduce sound, gravity, and head acceleration stimuli to the brain. Hearing and balance rely on mechanosensation, the fastest sensory signals transmitted to the brain. The mechanoelectrical transducer (MET) channel is the entryway for the sound-balance-brain interface, but the channel-complex composition is not entirely known. Here, we report that the mouse utilizes Piezo1 (Pz1) and Piezo2 (Pz2) isoforms as MET-complex components. The Pz channels, expressed in HC stereocilia, and cell lines are co-localized and co-assembled with MET complex partners...
January 16, 2024: Nature Communications
https://read.qxmd.com/read/38194445/lhfpl5-is-a-key-element-in-force-transmission-from-the-tip-link-to-the-hair-cell-mechanotransducer-channel
#17
JOURNAL ARTICLE
Maryline Beurg, Evan Travis Schwalbach, Robert Fettiplace
During auditory transduction, sound-evoked vibrations of the hair cell stereociliary bundles open mechanotransducer (MET) ion channels via tip links extending from one stereocilium to its neighbor. How tension in the tip link is delivered to the channel is not fully understood. The MET channel comprises a pore-forming subunit, transmembrane channel-like protein (TMC1 or TMC2), aided by several accessory proteins, including LHFPL5 (lipoma HMGIC fusion partner-like 5). We investigated the role of LHFPL5 in transduction by comparing MET channel activation in outer hair cells of Lhfpl5-/- knockout mice with those in Lhfpl5+/- heterozygotes...
January 16, 2024: Proceedings of the National Academy of Sciences of the United States of America
https://read.qxmd.com/read/38162163/novel-fermt3-and-ptprq-mutations-associated-with-leukocyte-adhesion-deficiency-iii-and-sensorineural-hearing-loss
#18
JOURNAL ARTICLE
Gabriela de Toledo Passos Candelaria, Alexandre de A Antunes, Antonio C Pastorino, Mayra de B Dorna, Evelin A Zanardo, Alexandre T Dias, Sofia M M Sugayama, Vicente Odone-Filho, Leslie D Kulikowski, Marlene P Garanito
Leukocyte adhesion deficiency-III (LAD-III) is a rare genetic disease caused by defective integrin activation in hematopoietic cells due to mutations in the FERMT3 gene. The PTPRQ gene encodes the protein tyrosine phosphatase receptor Q and is essential for the normal maturation and function of hair bundle in the cochlea. Homozygous PTPRQ mutations impair the stereocilia in hair cells which lead to nonsyndromic sensorineural hearing loss (SNHL) with vestibular dysfunction. Here, we report two novel pathogenic homozygous mutations found in two genes, FERMT3 and PTPRQ , in a Brazilian patient with LAD-III and SNHL, which may develop our understanding of the phenotype-genotype correlation and prognosis of patients with these rare diseases...
December 2023: Journal of Pediatric Genetics
https://read.qxmd.com/read/38108225/estrogen-as-a-guardian-of-auditory-health-tsp1-cd47-axis-regulation-and-noise-induced-hearing-loss
#19
JOURNAL ARTICLE
X Wang, H Zheng, B Yang, M Zu, Z Wang, J Zhang, F Zheng, M Yang, M C F Tong, L Zhao, W Bai
OBJECTIVES: This study aimed to analyze the role of estrogen in noise-induced hearing loss (NIHL) and uncover underlying mechanisms. METHODS: An ovariectomized Sprague-Dawley rat model (OVX) was constructed to investigate the hearing threshold and auditory latency before and after noise exposure using the auditory brainstem response (ABR) test. The morphological changes were assessed using immunofluorescence, scanning electron microscopy and transmission electron microscopy...
December 18, 2023: Climacteric: the Journal of the International Menopause Society
https://read.qxmd.com/read/38068993/the-relevance-of-autophagy-within-inner-ear-in-baseline-conditions-and-tinnitus-related-syndromes
#20
REVIEW
Gloria Lazzeri, Francesca Biagioni, Michela Ferrucci, Stefano Puglisi-Allegra, Paola Lenzi, Carla Letizia Busceti, Francesco Giannessi, Francesco Fornai
Tinnitus is the perception of noise in the absence of acoustic stimulation (phantom noise). In most patients suffering from chronic peripheral tinnitus, an alteration of outer hair cells (OHC) starting from the stereocilia (SC) occurs. This is common following ototoxic drugs, sound-induced ototoxicity, and acoustic degeneration. In all these conditions, altered coupling between the tectorial membrane (TM) and OHC SC is described. The present review analyzes the complex interactions involving OHC and TM. These need to be clarified to understand which mechanisms may underlie the onset of tinnitus and why the neuropathology of chronic degenerative tinnitus is similar, independent of early triggers...
November 23, 2023: International Journal of Molecular Sciences
keyword
keyword
166691
1
2
Fetch more papers »
Fetching more papers... Fetching...
Remove bar
Read by QxMD icon Read
×

Save your favorite articles in one place with a free QxMD account.

×

Search Tips

Use Boolean operators: AND/OR

diabetic AND foot
diabetes OR diabetic

Exclude a word using the 'minus' sign

Virchow -triad

Use Parentheses

water AND (cup OR glass)

Add an asterisk (*) at end of a word to include word stems

Neuro* will search for Neurology, Neuroscientist, Neurological, and so on

Use quotes to search for an exact phrase

"primary prevention of cancer"
(heart or cardiac or cardio*) AND arrest -"American Heart Association"

We want to hear from doctors like you!

Take a second to answer a survey question.