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inner ear hair cell

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https://www.readbyqxmd.com/read/28230212/establishment-of-planar-cell-polarity-is-coupled-to-regional-cell-cycle-exit-and-cell-differentiation-in-the-mouse-utricle
#1
Xiaoyu Yang, Xiaoqing Qian, Rui Ma, Xinwei Wang, Juanmei Yang, Wenwei Luo, Ping Chen, Fanglu Chi, Dongdong Ren
Sensory hair cells are coordinately oriented within each inner ear sensory organ to exhibit a particular form of planar cell polarity (PCP) necessary for mechanotransduction. However, the developmental events associated with establishing PCP in the vestibule are unclear, hindering data interpretation and employment of the vestibule for PCP studies. Herein, we investigated PCP of the mouse vestibular organs. We further characterised cell cycle exit, cell differentiation, and PCP establishment in the utricle...
February 23, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28228581/spaceflight-induced-synaptic-modifications-within-hair-cells-of-the-mammalian-utricle
#2
David R Sultemeier, Kristel R Choy, Felix E Schweizer, Larry F Hoffman
Exposure to the microgravity conditions of spaceflight alleviates the load normally imposed by the Earth's gravitational field upon the inner ear utricular epithelia. Previous ultrastructural investigations showed that spaceflight induced an increase in synapse density within hair cells of the rat utricle. However, the utricle exhibits broad physiologic heterogeneity across different epithelial regions, and it is unknown whether capabilities for synaptic plasticity generalize to hair cells across its topography...
February 22, 2017: Journal of Neurophysiology
https://www.readbyqxmd.com/read/28224282/micrornas-effective-elements-in-ear-related-diseases-and-hearing-loss
#3
REVIEW
Mohammad-Reza Mahmoudian-Sani, Ameneh Mehri-Ghahfarrokhi, Fereshteh Ahmadinejad, Morteza Hashemzadeh-Chaleshtori, Massoud Saidijam, Mohammad-Saeid Jami
miRNAs are important factors for post-transcriptional process that controls gene expression at mRNA level. Various biological processes, including growth and differentiation, are regulated by miRNAs. miRNAs have been demonstrated to play an essential role in development and progression of hearing loss. Nowadays, miRNAs are known as critical factors involved in different physiological, biological, and pathological processes, such as gene expression, progressive sensorineural hearing loss, age-related hearing loss, noise-induced hearing loss, cholesteatoma, schwannomas, and inner ear inflammation...
February 21, 2017: European Archives of Oto-rhino-laryngology
https://www.readbyqxmd.com/read/28223157/dose-dependent-cochlear-and-vestibular-toxicity-of-trans-tympanic-cisplatin-in-the-rat
#4
Angela Callejo, Amandine Durochat, Stéphanie Bressieux, Aurélie Saleur, Christian Chabbert, Ivan Domènech Juan, Jordi Llorens, Sophie Gaboyard-Niay
In vivo studies are needed to study cisplatin ototoxicity and to evaluate candidate protective treatments. Rats and mice are the preferred species for toxicological and pharmacological pre-clinical research, but systemic administration of cisplatin causes high morbidity in these species. We hypothesized that trans-tympanic administration of cisplatin would provide a good model for studying its auditory and vestibular toxicity in the rat. Cisplatin was administered by the trans-tympanic route in one ear (50μl, 0...
February 14, 2017: Neurotoxicology
https://www.readbyqxmd.com/read/28220837/a-novel-role-of-the-organizer-gene-goosecoid-as-an-inhibitor-of-wnt-pcp-mediated-convergent-extension-in-xenopus-and-mouse
#5
Bärbel Ulmer, Melanie Tingler, Sabrina Kurz, Markus Maerker, Philipp Andre, Dina Mönch, Marina Campione, Kirsten Deißler, Mark Lewandoski, Thomas Thumberger, Axel Schweickert, Abraham Fainsod, Herbert Steinbeißer, Martin Blum
Goosecoid (Gsc) expression marks the primary embryonic organizer in vertebrates and beyond. While functions have been assigned during later embryogenesis, the role of Gsc in the organizer has remained enigmatic. Using conditional gain-of-function approaches in Xenopus and mouse to maintain Gsc expression in the organizer and along the axial midline, neural tube closure defects (NTDs) arose and dorsal extension was compromised. Both phenotypes represent convergent extension (CE) defects, arising from impaired Wnt/planar cell polarity (PCP) signaling...
February 21, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28196606/imaging-and-cell-count-in-cleared-intact-cochlea-in-the-mongolian-gerbil-using-laser-scanning-confocal-microscopy
#6
M Risoud, J Sircoglou, G Dedieu, M Tardivel, C Vincent, N-X Bonne
OBJECTIVES: To draw up a clearing protocol for Mongolian gerbil cochlea, and to assess the feasibility of quantifying and analyzing 3D cell architecture in the transparent cochleae. MATERIALS AND METHODS: Freshly dissected inner ears were prepared on a 13-day protocol: fixation, microdissection, post-fixation, decalcification, pretreatment (signal enhancement, permeabilization and blocking), fluorescent labeling (indirect immunolabeling and direct labeling), dehydration, clearing in Spalteholz solution (MSBB: methyl salicylate and benzyl benzoate) and mounting...
February 9, 2017: European Annals of Otorhinolaryngology, Head and Neck Diseases
https://www.readbyqxmd.com/read/28181574/indispensable-role-of-mdm2-p53-interaction-during-the-embryonic-and-postnatal-inner-ear-development
#7
M Laos, M Sulg, A Herranen, T Anttonen, U Pirvola
p53 is a key component of a signaling network that protects cells against various stresses. As excess p53 is detrimental to cells, its levels are tightly controlled by several mechanisms. The E3 ubiquitin ligase Mdm2 is a major negative regulator of p53. The significance of balanced p53 levels in normal tissues, at different stages of lifetime, is poorly understood. We have studied in vivo how the disruption of Mdm2/p53 interaction affects the early-embryonic otic progenitor cells and their descendants, the auditory supporting cells and hair cells...
February 9, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28181545/gfi1-cre-mice-have-early-onset-progressive-hearing-loss-and-induce-recombination-in-numerous-inner-ear-non-hair-cells
#8
Maggie Matern, Sarath Vijayakumar, Zachary Margulies, Beatrice Milon, Yang Song, Ran Elkon, Xiaoyu Zhang, Sherri M Jones, Ronna Hertzano
Studies of developmental and functional biology largely rely on conditional expression of genes in a cell type-specific manner. Therefore, the importance of specificity and lack of inherent phenotypes for Cre-driver animals cannot be overemphasized. The Gfi1(Cre) mouse is commonly used for conditional hair cell-specific gene deletion/reporter gene activation in the inner ear. Here, using immunofluorescence and flow cytometry, we show that the Gfi1(Cre) mice produce a pattern of recombination that is not strictly limited to hair cells within the inner ear...
February 9, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28179572/spectrin-%C3%AE-v-adaptive-mutations-and-changes-in-subcellular-location-correlate-with-emergence-of-hair-cell-electromotility-in-mammalians
#9
Matteo Cortese, Samantha Papal, Francisco Pisciottano, Ana Belén Elgoyhen, Jean-Pierre Hardelin, Christine Petit, Lucia Florencia Franchini, Aziz El-Amraoui
The remarkable hearing capacities of mammals arise from various evolutionary innovations. These include the cochlear outer hair cells and their singular feature, somatic electromotility, i.e., the ability of their cylindrical cell body to shorten and elongate upon cell depolarization and hyperpolarization, respectively. To shed light on the processes underlying the emergence of electromotility, we focused on the βV giant spectrin, a major component of the outer hair cells' cortical cytoskeleton. We identified strong signatures of adaptive evolution at multiple sites along the spectrin-βV amino acid sequence in the lineage leading to mammals, together with substantial differences in the subcellular location of this protein between the frog and the mouse inner ear hair cells...
February 8, 2017: Proceedings of the National Academy of Sciences of the United States of America
https://www.readbyqxmd.com/read/28165504/implementation-of-a-method-to-visualize-noise-induced-hearing-loss-in-mass-stranded-cetaceans
#10
Maria Morell, Andrew Brownlow, Barry McGovern, Stephen A Raverty, Robert E Shadwick, Michel André
Assessment of the impact of noise over-exposure in stranded cetaceans is challenging, as the lesions that lead to hearing loss occur at the cellular level and inner ear cells are very sensitive to autolysis. Distinguishing ante-mortem pathology from post-mortem change has been a major constraint in diagnosing potential impact. Here, we outline a methodology applicable to the detection of noise-induced hearing loss in stranded cetaceans. Inner ears from two mass strandings of long-finned pilot whales in Scotland were processed for scanning electron microscopy observation...
February 6, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28165476/gene-therapy-restores-auditory-and-vestibular-function-in-a-mouse-model-of-usher-syndrome-type-1c
#11
Bifeng Pan, Charles Askew, Alice Galvin, Selena Heman-Ackah, Yukako Asai, Artur A Indzhykulian, Francine M Jodelka, Michelle L Hastings, Jennifer J Lentz, Luk H Vandenberghe, Jeffrey R Holt, Gwenaëlle S Géléoc
Because there are currently no biological treatments for hearing loss, we sought to advance gene therapy approaches to treat genetic deafness. We focused on Usher syndrome, a devastating genetic disorder that causes blindness, balance disorders and profound deafness, and studied a knock-in mouse model, Ush1c c.216G>A, for Usher syndrome type IC (USH1C). As restoration of complex auditory and balance function is likely to require gene delivery systems that target auditory and vestibular sensory cells with high efficiency, we delivered wild-type Ush1c into the inner ear of Ush1c c...
February 6, 2017: Nature Biotechnology
https://www.readbyqxmd.com/read/28165475/a-synthetic-aav-vector-enables-safe-and-efficient-gene-transfer-to-the-mammalian-inner-ear
#12
Lukas D Landegger, Bifeng Pan, Charles Askew, Sarah J Wassmer, Sarah D Gluck, Alice Galvin, Ruth Taylor, Andrew Forge, Konstantina M Stankovic, Jeffrey R Holt, Luk H Vandenberghe
Efforts to develop gene therapies for hearing loss have been hampered by the lack of safe, efficient, and clinically relevant delivery modalities. Here we demonstrate the safety and efficiency of Anc80L65, a rationally designed synthetic vector, for transgene delivery to the mouse cochlea. Ex vivo transduction of mouse organotypic explants identified Anc80L65 from a set of other adeno-associated virus (AAV) vectors as a potent vector for the cochlear cell targets. Round window membrane injection resulted in highly efficient transduction of inner and outer hair cells in mice, a substantial improvement over conventional AAV vectors...
February 6, 2017: Nature Biotechnology
https://www.readbyqxmd.com/read/28159525/celsr1-coordinates-the-planar-polarity-of-vestibular-hair-cells-during-inner-ear-development
#13
Jeremy S Duncan, Michelle L Stoller, Andrew F Francl, Fadel Tissir, Danelle Devenport, Michael R Deans
Vestibular hair cells of the inner ear are specialized receptors that detect mechanical stimuli from gravity and motion via the deflection of a polarized bundle of stereocilia located on their apical cell surfaces. The orientation of stereociliary bundles is coordinated between neighboring cells by core PCP proteins including the large adhesive G-protein coupled receptor Celsr1. We show that mice lacking Celsr1 have vestibular behavioral phenotypes including circling. In addition, we show that Celsr1 is asymmetrically distributed at cell boundaries between hair cells and neighboring supporting cells in the developing vestibular and auditory sensory epithelia...
February 1, 2017: Developmental Biology
https://www.readbyqxmd.com/read/28154149/the-coupling-between-ca2-channels-and-the-exocytotic-ca2-sensor-at-hair-cell-ribbon-synapses-varies-tonotopically-along-the-mature-cochlea
#14
Stuart L Johnson, Jennifer Olt, Soyoun Cho, Henrique von Gersdorff, Walter Marcotti
: The cochlea processes auditory signals over a wide range of frequencies and intensities. However, the transfer characteristics at hair cell ribbon synapses are still poorly understood at different frequency locations along the cochlea. Using recordings from mature gerbils, we report here a surprisingly strong block of exocytosis by the slow Ca(2+) buffer EGTA (10 mM) in basal hair cells tuned to high frequencies (∼30 kHz). In addition, using recordings from gerbil, mouse and bullfrog auditory organs, we find that the spatial coupling between Ca(2+) influx and exocytosis changes from nanodomain in low-frequency tuned hair cells (<∼2 kHz) to progressively more microdomain in high-frequency cells (>∼2 kHz)...
February 2, 2017: Journal of Neuroscience: the Official Journal of the Society for Neuroscience
https://www.readbyqxmd.com/read/28118374/dynamic-expression-of-sox2-gata3-and-prox1-during-primary-auditory-neuron-development-in-the-mammalian-cochlea
#15
Koji Nishimura, Teppei Noda, Alain Dabdoub
Primary auditory neurons (PANs) connect cochlear sensory hair cells in the mammalian inner ear to cochlear nucleus neurons in the brainstem. PANs develop from neuroblasts delaminated from the proneurosensory domain of the otocyst and keep maturing until the onset of hearing after birth. There are two types of PANs: type I, which innervate the inner hair cells (IHCs), and type II, which innervate the outer hair cells (OHCs). Glial cells surrounding these neurons originate from neural crest cells and migrate to the spiral ganglion...
2017: PloS One
https://www.readbyqxmd.com/read/28115485/ick-ciliary-kinase-is-essential-for-planar-cell-polarity-formation-in-inner-ear-hair-cells-and-hearing-function
#16
Shio Okamoto, Taro Chaya, Yoshihiro Omori, Ryusuke Kuwahara, Shun Kubo, Hirofumi Sakaguchi, Takahisa Furukawa
: Cellular asymmetries play crucial roles in development and organ function. The planar cell polarity (PCP) signaling pathway is involved in the establishment of cellular asymmetry within the plane of a cell sheet. Inner ear sensory hair cells, which have several rows of staircase-like stereocilia and one kinocilium located at the vertex of the stereocilia protruding from the apical surface of each hair cell, exhibit a typical form of PCP. While connections between cilia and PCP signaling in vertebrate development have been reported, their precise nature is not well understood...
January 23, 2017: Journal of Neuroscience: the Official Journal of the Society for Neuroscience
https://www.readbyqxmd.com/read/28109437/analysis-of-the-proteome-of-hair-cell-stereocilia-by-mass-spectrometry
#17
J F Krey, P A Wilmarth, L L David, P G Barr-Gillespie
Characterization of proteins that mediate mechanotransduction by hair cells, the sensory cells of the inner ear, is hampered by the scarcity of these cells and their sensory organelle, the hair bundle. Mass spectrometry, with its high sensitivity and identification precision, is the ideal method for determining which proteins are present in bundles and what proteins they interact with. We describe here the isolation of mouse hair bundles, as well as preparation of bundle protein samples for mass spectrometry...
2017: Methods in Enzymology
https://www.readbyqxmd.com/read/28105691/the-alcohol-sensitive-period-during-early-octavolateral-organ-development-in-zebrafish-danio-rerio
#18
Lilliann Y Zamora, Kayla C Miguel, Zhongmin Lu
Fetal alcohol exposure can cause Fetal Alcohol Spectrum Disorders (FASD), completely preventable developmental disabilities characterized by permanent birth defects. However, specific gestational timing when developing organs are most sensitive to alcohol exposure is unclear. In this study, we examined the temporal effects of embryonic alcohol exposure on octavolateral organs in zebrafish (Danio rerio), including inner ears and lateral line neuromasts that function in hearing, balance, and hydrodynamic detection, respectively...
January 20, 2017: Journal of Neuroscience Research
https://www.readbyqxmd.com/read/28087419/cochlear-synaptopathy-in-acquired-sensorineural-hearing-loss-manifestations-and-mechanisms
#19
REVIEW
M Charles Liberman, Sharon G Kujawa
Common causes of hearing loss in humans - exposure to loud noise or ototoxic drugs and aging - often damage sensory hair cells, reflected as elevated thresholds on the clinical audiogram. Recent studies in animal models suggest, however, that well before this overt hearing loss can be seen, a more insidious, but likely more common, process is taking place that permanently interrupts synaptic communication between sensory inner hair cells and subsets of cochlear nerve fibers. The silencing of affected neurons alters auditory information processing, whether accompanied by threshold elevations or not, and is a likely contributor to a variety of perceptual abnormalities, including speech-in-noise difficulties, tinnitus and hyperacusis...
January 10, 2017: Hearing Research
https://www.readbyqxmd.com/read/28039372/global-analysis-of-protein-expression-of-inner-ear-hair-cells
#20
Ann E Hickox, Ann C Y Wong, Kwang Pak, Chelsee Strojny, Miguel Ramirez, John R Yates, Allen F Ryan, Jeffrey N Savas
: The mammalian inner ear subserves auditory and vestibular sensations via highly specialized cells and proteins. Sensory receptor hair cells (HCs) are necessary for transducing mechanical inputs and stimulating sensory neurons by employing a host of known and yet unknown protein machinery. To understand the protein composition of these unique post-mitotic cells, in which irreversible protein degradation or damage can lead to impaired hearing and balance, we analyzed inner ear samples by tandem mass spectrometry to generate an unbiased, shotgun-proteomics view of protein identities and abundances...
December 30, 2016: Journal of Neuroscience: the Official Journal of the Society for Neuroscience
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