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cochlear hair cell

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https://www.readbyqxmd.com/read/28929130/cytoskeletal-stability-in-the-auditory-organ-in-vivo-rhoa-is-dispensable-for-wound-healing-but-essential-for-hair-cell-development
#1
Tommi Anttonen, Ilya Belevich, Maarja Laos, Anni Herranen, Eija Jokitalo, Cord Brakebusch, Ulla Pirvola
Wound healing in the inner ear sensory epithelia is performed by the apical domains of supporting cells (SCs). Junctional F-actin belts of SCs are thin during development but become exceptionally thick during maturation. The functional significance of the thick belts is not fully understood. We have studied the role of F-actin belts during wound healing in the developing and adult cochlea of mice in vivo. We show that the thick belts serve as intracellular scaffolds that preserve the positions of surviving cells in the cochlear sensory epithelium...
September 2017: ENeuro
https://www.readbyqxmd.com/read/28928635/d-tubocurarine-and-berbamine-alkaloids-that-are-permeant-blockers-of-the-hair-cell-s-mechano-electrical-transducer-channel-and-protect-from-aminoglycoside-toxicity
#2
Nerissa K Kirkwood, Molly O'Reilly, Marco Derudas, Emma J Kenyon, Rosemary Huckvale, Sietse M van Netten, Simon E Ward, Guy P Richardson, Corné J Kros
Aminoglycoside antibiotics are widely used for the treatment of life-threatening bacterial infections, but cause permanent hearing loss in a substantial proportion of treated patients. The sensory hair cells of the inner ear are damaged following entry of these antibiotics via the mechano-electrical transducer (MET) channels located at the tips of the hair cell's stereocilia. d-Tubocurarine (dTC) is a MET channel blocker that reduces the loading of gentamicin-Texas Red (GTTR) into rat cochlear hair cells and protects them from gentamicin treatment...
2017: Frontiers in Cellular Neuroscience
https://www.readbyqxmd.com/read/28928030/the-histone-deacetylase-inhibitor-sodium-butyrate-protects-against-noise-induced-hearing-loss-in-guinea-pigs
#3
Deng-Hua Yang, Jing Xie, Ke Liu, Zhe Peng, Jing-Ying Guo, Shu-Kui Yu, Guo-Peng Wang, Shu-Sheng Gong
Noise-induced hearing loss (NIHL) severely impacts the quality of life of affected individuals. Oxidative stress resulting from noise exposure is a significant cause of NIHL. Although histone deacetylase (HDAC) inhibitors were shown to protect against NIHL, the underlying mechanism remains unclear, and it is not known how they act on noise-induced oxidative stress. In the current study, we investigated the expression levels of acetyl-histone H3 (Lys9) (H3-AcK9), histone deacetylase 1 (HDAC1), and 3-nitrotyrosine (3-NT), an oxidative stress marker, in a guinea pig model of NIHL using immunohistology and Western blotting...
September 16, 2017: Neuroscience Letters
https://www.readbyqxmd.com/read/28924861/role-of-bdnf-and-neurotrophic-receptors-in-human-inner-ear-development
#4
L Johnson Chacko, M J F Blumer, E Pechriggl, H Rask-Andersen, W Dietl, A Haim, H Fritsch, R Glueckert, J Dudas, A Schrott-Fischer
The expression patterns of the neurotrophin, brain-derived neurotrophic factor, BDNF, and the neurotrophic receptors-p75NTR and Trk receptors-in the developing human fetal inner ear between the gestational weeks (GW) 9 to 12 are examined via in situ hybridization and immunohistochemistry. BDNF mRNA expression was highest in the cochlea at GW 9 but declined in the course of development. In contrast to embryonic murine specimens, a decline in BDNF expression from the apical to the basal turn of the cochlea could not be observed...
September 19, 2017: Cell and Tissue Research
https://www.readbyqxmd.com/read/28900799/peroxynitrite-induces-apoptosis-of-mouse-cochlear-hair-cells-via-a-caspase-independent-pathway-in-vitro
#5
Zhixin Cao, Qianqian Yang, Haiyan Yin, Qi Qi, Hongrui Li, Gaoying Sun, Hongliang Wang, Wenwen Liu, Jianfeng Li
Peroxynitrite (ONOO(-)) is a potent and versatile oxidant implicated in a number of pathophysiological processes. The present study was designed to investigate the effect of ONOO(-) on the cultured cochlear hair cells (HCs) of C57BL/6 mice in vitro as well as the possible mechanism underlying the action of such an oxidative stress. The in vitro primary cultured cochlear HCs were subjected to different concentrations of ONOO(-), then, the cell survival and morphological changes were examined by immunofluorescence and transmission electron microscopy (TEM), the apoptosis was determined by Terminal deoxynucleotidyl transferase dUNT nick end labeling (TUNEL) assay, the mRNA expressions of Caspase-3, Caspase-8, Caspase-9, Apaf1, Bcl-2, and Bax were analyzed by RT-PCR, and the protein expressions of Caspase-3 and AIF were assessed by immunofluorescence...
September 12, 2017: Apoptosis: An International Journal on Programmed Cell Death
https://www.readbyqxmd.com/read/28893864/the-beach-protein-lrba-is-required-for-hair-bundle-maintenance-in-cochlear-hair-cells-and-for-hearing
#6
Christian Vogl, Tanvi Butola, Natja Haag, Torben J Hausrat, Michael G Leitner, Michel Moutschen, Philippe P Lefèbvre, Carsten Speckmann, Lillian Garrett, Lore Becker, Helmut Fuchs, Martin Hrabe de Angelis, Sandor Nietzsche, Michael M Kessels, Dominik Oliver, Matthias Kneussel, Manfred W Kilimann, Nicola Strenzke
Lipopolysaccharide-responsive beige-like anchor protein (LRBA) belongs to the enigmatic class of BEACH domain-containing proteins, which have been attributed various cellular functions, typically involving intracellular protein and membrane transport processes. Here, we show that LRBA deficiency in mice leads to progressive sensorineural hearing loss. In LRBA knockout mice, inner and outer hair cell stereociliary bundles initially develop normally, but then partially degenerate during the second postnatal week...
September 11, 2017: EMBO Reports
https://www.readbyqxmd.com/read/28892484/six1-is-essential-for-differentiation-and-patterning-of-the-mammalian-auditory-sensory-epithelium
#7
Ting Zhang, Jinshu Xu, Pascal Maire, Pin-Xian Xu
The organ of Corti in the cochlea is a two-cell layered epithelium: one cell layer of mechanosensory hair cells that align into one row of inner and three rows of outer hair cells interdigitated with one cell layer of underlying supporting cells along the entire length of the cochlear spiral. These two types of epithelial cells are derived from common precursors in the four- to five-cell layered primordium and acquire functionally important shapes during terminal differentiation through the thinning process and convergent extension...
September 2017: PLoS Genetics
https://www.readbyqxmd.com/read/28892270/biomimetic-artificial-basilar-membranes-for-next-generation-cochlear-implants
#8
REVIEW
Jongmoon Jang, Jeong Hun Jang, Hongsoo Choi
Patients with sensorineural hearing loss can recover their hearing using a cochlear implant (CI). However, there is a need to develop next-generation CIs to overcome the limitations of conventional CIs caused by extracorporeal devices. Recently, artificial basilar membranes (ABMs) are actively studied for next-generation CIs. The ABM is an acoustic transducer that mimics the mechanical frequency selectivity of the BM and acoustic-to-electrical energy conversion of hair cells. This paper presents recent progress in biomimetic ABMs...
September 11, 2017: Advanced Healthcare Materials
https://www.readbyqxmd.com/read/28880884/two-passive-mechanical-conditions-modulate-power-generation-by-the-outer-hair-cells
#9
Yanju Liu, Sheryl M Gracewski, Jong-Hoon Nam
In the mammalian cochlea, small vibrations of the sensory epithelium are amplified due to active electro-mechanical feedback of the outer hair cells. The level of amplification is greater in the base than in the apex of the cochlea. Theoretical studies have used longitudinally varying active feedback properties to reproduce the location-dependent amplification. The active feedback force has been considered to be proportional to the basilar membrane displacement or velocity. An underlying assumption was that organ of Corti mechanics are governed by rigid body kinematics...
September 2017: PLoS Computational Biology
https://www.readbyqxmd.com/read/28874607/no-sensitive-guanylate-cyclase-isoforms-no-gc1-and-no-gc2-contribute-to-noise-induced-inner-hair-cell-synaptopathy
#10
Dorit Möhrle, Katrin Reimann, Steffen Wolter, Markus Wolters, Ksenya Varakina, Evanthia Mergia, Nicole Eichert, Hyun-Soon Geisler, Peter Sandner, Peter Ruth, Andreas Friebe, Robert Feil, Ulrike Zimmermann, Doris Koesling, Marlies Knipper, Lukas Rüttiger
Nitric oxide (NO) activates the NO-sensitive soluble guanylate cyclase (NO-GC, sGC) and triggers intracellular signaling pathways involving cGMP. For survival of cochlear hair cells and preservation of hearing, NO-mediated cascades have both protective and detrimental potential. Here we examine the cochlear function of mice lacking one of the two NO-sensitive guanylate cyclase isoforms [NO-GC1 knockout (KO) or NO-GC2 KO]. The deletion of NO-GC1 or NO-GC2 did not influence electromechanical outer hair cell (OHC) properties, as measured by distortion product otoacoustic emissions, neither before nor after noise exposure, nor were click- or noise-burst-evoked auditory brainstem response thresholds different from controls...
October 2017: Molecular Pharmacology
https://www.readbyqxmd.com/read/28870992/cell-migration-intercalation-and-growth-regulates-mammalian-cochlear-extension
#11
Elizabeth Carroll Driver, Amy Northrop, Matthew W Kelley
Developmental remodeling of the sensory epithelium of the cochlea is required for the formation of an elongated, tonotopically organized auditory organ, but the cellular processes that mediate these events are largely unknown. Therefore, we used both morphological assessments of cellular rearrangements and time-lapse imaging to visualize cochlear remodeling. Analysis of cell redistribution showed that the cochlea extends through a combination of radial intercalation and cell growth. Live imaging demonstrated that concomitant cellular intercalation results in a brief period of epithelial convergence, although subsequent changes in cell size lead to medial-lateral spreading...
September 4, 2017: Development
https://www.readbyqxmd.com/read/28867994/an-antioxidant-screen-identifies-candidates-for-protection-of-cochlear-hair-cells-from-gentamicin-toxicity
#12
Volker Noack, Kwang Pak, Rahul Jalota, Arwa Kurabi, Allen F Ryan
Reactive oxygen species are important elements in ototoxic damage to hair cells (HCs), appearing early in the damage process. Higher levels of natural antioxidants are positively correlated with resistance to ototoxins and many studies have shown that exogenous antioxidants can protect HCs from damage. While a very wide variety of antioxidants with different characteristics and intracellular targets exist, most ototoxicity studies have focused upon one or a few well-characterized compounds. Relatively little research has attempted to determine the comparative efficacy of large variety of different antioxidants...
2017: Frontiers in Cellular Neuroscience
https://www.readbyqxmd.com/read/28855028/fgf-signaling-diverse-roles-during-cochlear-development
#13
Michael Ebeid, Sung-Ho Huh
Mammalian inner ear comprises of six sensory organs; cochlea, utricle, saccule, and three semicircular canals. The cochlea contains sensory epithelium known as the organ of Corti which senses sound through mechanosensory hair cells. Mammalian inner ear undergoes series of morphogenesis during development by beginning thickening of ectoderm nearby hindbrain. These events require tight regulation of multiple signaling cascades including FGF, Wnt, Notch and Bmp signaling. In this review, we will discuss the role of newly emerging signaling, FGF signaling, for its roles required for cochlear development...
August 31, 2017: BMB Reports
https://www.readbyqxmd.com/read/28852025/intravenous-raav2-9-injection-for-murine-cochlear-gene-delivery
#14
Seiji B Shibata, Hidekane Yoshimura, Paul T Ranum, Alexander T Goodwin, Richard J H Smith
Gene therapy for genetic deafness is a promising approach by which to prevent hearing loss or to restore hearing after loss has occurred. Although a variety of direct approaches to introduce viral particles into the inner ear have been described, presumed physiological barriers have heretofore precluded investigation of systemic gene delivery to the cochlea. In this study, we sought to characterize systemic delivery of a rAAV2/9 vector as a non-invasive means of cochlear transduction. In wild-type neonatal mice (postnatal day 0-1), we show that intravenous injection of rAAV2/9 carrying an eGFP-reporter gene results in binaural transduction of inner hair cells, spiral ganglion neurons and vestibular hair cells...
August 29, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28840850/classification-and-current-management-of-inner-ear-malformations
#15
Levent Sennaroğlu, Münir Demir Bajin
Morphologically congenital sensorineural hearing loss can be investigated under two categories. Majority of the congenital hearing loss (80%) are membranous malformations. Here the pathology involves inner ear hair cells. There is no gross bony abnormality and therefore, in these cases, high resolution computerized tomography and MRI of the temporal bone reveal normal findings. Remaining 20% have various malformations involving the bony labyrinth and therefore, can be radiologically demonstrated by CT and MRI...
August 25, 2017: Balkan Medical Journal
https://www.readbyqxmd.com/read/28837644/prickle1-regulates-neurite-outgrowth-of-apical-spiral-ganglion-neurons-but-not-hair-cell-polarity-in-the-murine-cochlea
#16
Tian Yang, Jennifer Kersigo, Shu Wu, Bernd Fritzsch, Alexander G Bassuk
In the mammalian organ of Corti (OC), the stereocilia on the apical surface of hair cells (HCs) are uniformly organized in a neural to abneural axis (or medial-laterally). This organization is regulated by planar cell polarity (PCP) signaling. Mutations of PCP genes, such as Vangl2, Dvl1/2, Celsr1, and Fzd3/6, affect the formation of HC orientation to varying degrees. Prickle1 is a PCP signaling gene that belongs to the prickle / espinas / testin family. Prickle1 protein is shown to be asymmetrically localized in the HCs of the OC, and this asymmetric localization is associated with loss of PCP in Smurf mutants, implying that Prickle1 is involved in HC PCP development in the OC...
2017: PloS One
https://www.readbyqxmd.com/read/28835534/local-gene-therapy-durably-restores-vestibular-function-in-a-mouse-model-of-usher-syndrome-type-1g
#17
Alice Emptoz, Vincent Michel, Andrea Lelli, Omar Akil, Jacques Boutet de Monvel, Ghizlene Lahlou, Anaïs Meyer, Typhaine Dupont, Sylvie Nouaille, Elody Ey, Filipa Franca de Barros, Mathieu Beraneck, Didier Dulon, Jean-Pierre Hardelin, Lawrence Lustig, Paul Avan, Christine Petit, Saaid Safieddine
Our understanding of the mechanisms underlying inherited forms of inner ear deficits has considerably improved during the past 20 y, but we are still far from curative treatments. We investigated gene replacement as a strategy for restoring inner ear functions in a mouse model of Usher syndrome type 1G, characterized by congenital profound deafness and balance disorders. These mice lack the scaffold protein sans, which is involved both in the morphogenesis of the stereociliary bundle, the sensory antenna of inner ear hair cells, and in the mechanoelectrical transduction process...
September 5, 2017: Proceedings of the National Academy of Sciences of the United States of America
https://www.readbyqxmd.com/read/28832620/a-novel-splice-site-mutation-of-myosin-vi-in-mice-leads-to-stereociliary-fusion-caused-by-disruption-of-actin-networks-in-the-apical-region-of-inner-ear-hair-cells
#18
Yuta Seki, Yuki Miyasaka, Sari Suzuki, Kenta Wada, Shumpei P Yasuda, Kunie Matsuoka, Yasuhiro Ohshiba, Kentaro Endo, Rie Ishii, Hiroshi Shitara, Shin-Ichiro Kitajiri, Naomi Nakagata, Hirohide Takebayashi, Yoshiaki Kikkawa
An unconventional myosin encoded by the myosin VI gene (MYO6) contributes to hearing loss in humans. Homozygous mutations of MYO6 result in nonsyndromic profound congenital hearing loss, DFNB37. Kumamoto shaker/waltzer (ksv) mice harbor spontaneous mutations, and homozygous mutants exhibit congenital defects in balance and hearing caused by fusion of the stereocilia. We identified a Myo6c.1381G>A mutation that was found to be a p.E461K mutation leading to alternative splicing errors in Myo6 mRNA in ksv mutants...
2017: PloS One
https://www.readbyqxmd.com/read/28832600/hpn-07-a-free-radical-spin-trapping-agent-protects-against-functional-cellular-and-electrophysiological-changes-in-the-cochlea-induced-by-acute-acoustic-trauma
#19
Donald Ewert, Ning Hu, Xiaoping Du, Wei Li, Matthew B West, Chul-Hee Choi, Robert Floyd, Richard D Kopke
Oxidative stress is considered a major cause of the structural and functional changes associated with auditory pathologies induced by exposure to acute acoustic trauma AAT). In the present study, we examined the otoprotective effects of 2,4-disulfophenyl-N-tert-butylnitrone (HPN-07), a nitrone-based free radical trap, on the physiological and cellular changes in the auditory system of chinchilla following a six-hour exposure to 4 kHz octave band noise at 105 dB SPL. HPN-07 has been shown to suppress oxidative stress in biological models of a variety of disorders...
2017: PloS One
https://www.readbyqxmd.com/read/28818524/loss-of-sestrin-2-potentiates-the-early-onset-of-age-related-sensory-cell-degeneration-in-the-cochlea
#20
Celia Zhang, Wei Sun, Ji Li, Binbin Xiong, Mitchell D Frye, Dalian Ding, Richard Salvi, Mi-Jung Kim, Shinichi Someya, Bo Hua Hu
Sestrin 2 (SESN2) is a stress-inducible protein that protects tissues from oxidative stress and delays the aging process. However, its role in maintaining the functional and structural integrity of the cochlea is largely unknown. Here, we report the expression of SESN2 protein in the sensory epithelium, particularly in hair cells. Using C57BL/6J mice, a mouse model of age-related cochlear degeneration, we observed a significant age-related reduction in SESN2 expression in cochlear tissues that was associated with early onset hearing loss and accelerated age-related sensory cell degeneration that progressed from the base toward the apex of the cochlea...
October 11, 2017: Neuroscience
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