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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/28925723/expression-patterns-of-members-of-the-isocitrate-dehydrogenase-gene-family-in-murine-inner-ear
#2
Y-R Kim, K-H Kim, S Lee, S-K Oh, J-W Park, K-Y Lee, J-I Baek, U-K Kim
Age-related hearing loss (ARHL) is characterized by an age-dependent decline of auditory function characterized by with loss of sensory hair cells, spiral ganglion neurons, and stria vascularis (SV) cells in the cochlea of the inner ear. Aging and age-related diseases result from accumulated oxidative damage caused by reactive oxygen species (ROS) generated by mitochondria. The isocitrate dehydrogenase (IDH) family includes three enzymes in human cells: IDH1, IDH2, and IDH3. Although all three enzymes catalyze the same enzymatic reaction, that is, oxidative decarboxylation of isocitrate to produce α-ketoglutarate, each IDH enzyme has unique features...
September 19, 2017: Biotechnic & Histochemistry: Official Publication of the Biological Stain Commission
https://www.readbyqxmd.com/read/28924861/role-of-bdnf-and-neurotrophic-receptors-in-human-inner-ear-development
#3
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/28923514/cell-polarity-and-planar-cell-polarity-pcp-in-spermatogenesis
#4
REVIEW
Haiqi Chen, Dolores D Mruk, Wing-Yee Lui, Chris K C Wong, Will M Lee, C Yan Cheng
In adult mammalian testes, spermatids, most notably step 17-19 spermatids in stage IV-VIII tubules, are aligned with their heads pointing toward the basement membrane and their tails toward the tubule lumen. On the other hand, these polarized spermatids also align across the plane of seminiferous epithelium, mimicking planar cell polarity (PCP) found in other hair cells in cochlea (inner ear). This orderly alignment of developing spermatids during spermiogenesis is important to support spermatogenesis, such that the maximal number of developing spermatids can be packed and supported by a fixed population of differentiated Sertoli cells in the limited space of the seminiferous epithelium in adult testes...
September 15, 2017: Seminars in Cell & Developmental Biology
https://www.readbyqxmd.com/read/28915323/hair-cell-transduction-tuning-and-synaptic-transmission-in-the-mammalian-cochlea
#5
Robert Fettiplace
Sound pressure fluctuations striking the ear are conveyed to the cochlea, where they vibrate the basilar membrane on which sit hair cells, the mechanoreceptors of the inner ear. Recordings of hair cell electrical responses have shown that they transduce sound via submicrometer deflections of their hair bundles, which are arrays of interconnected stereocilia containing the mechanoelectrical transducer (MET) channels. MET channels are activated by tension in extracellular tip links bridging adjacent stereocilia, and they can respond within microseconds to nanometer displacements of the bundle, facilitated by multiple processes of Ca2+-dependent adaptation...
September 12, 2017: Comprehensive Physiology
https://www.readbyqxmd.com/read/28892484/six1-is-essential-for-differentiation-and-patterning-of-the-mammalian-auditory-sensory-epithelium
#6
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/28880884/two-passive-mechanical-conditions-modulate-power-generation-by-the-outer-hair-cells
#7
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/28870992/cell-migration-intercalation-and-growth-regulates-mammalian-cochlear-extension
#8
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/28855028/fgf-signaling-diverse-roles-during-cochlear-development
#9
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/28853245/treatment-of-peripheral-vestibular-dysfunction-using-photobiomodulation
#10
Min Young Lee, Jai-Hwan Hyun, Myung-Whan Suh, Jin-Chul Ahn, Phil-Sang Chung, Jae Yun Jung, Chung Ku Rhee
Gentamicin, which is still used in modern medicine, is a known vestibular toxic agent, and various degrees of balance problems have been observed after exposure to this pharmacologic agent. Photobiomodulation is a candidate therapy for vertigo due to its ability to reach deep inner ear organs such as the cochlea. Previous reports have suggested that photobiomodulation can improve hearing and cochlea function. However, few studies have examined the effect of photobiomodulation on balance dysfunction. We used a rat model to mimic human vestibulopathy resulting from gentamicin treatment and evaluated the effect of photobiomodulation on vestibular toxicity...
August 2017: Journal of Biomedical Optics
https://www.readbyqxmd.com/read/28852025/intravenous-raav2-9-injection-for-murine-cochlear-gene-delivery
#11
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
#12
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/28837860/pomegranate-peel-extract-attenuates-d-galactose-induced-oxidative-stress-and-hearing-loss-by-regulating-pnuts-pp1-activity-in-the-mouse-cochlea
#13
Shuangyue Liu, Tao Xu, Xidi Wu, Yuhan Lin, Dongyan Bao, Yang Di, Tingting Ma, Yan Dang, Peili Jia, Jianqiao Xian, Aimei Wang, Yongxin Liu
Oxidative stress is considered to be a major contributor to age-related hearing loss (ARHL). Here, we investigated whether pomegranate peel extract (PPE) protected against hearing loss by decreased oxidative stress in the cochlea of D-galactose-induced accelerated aging mice. The aging mice exhibited an increase in hearing threshold shifts and hair cells loss, which were improved in the PPE-treated aging mice. The aging mice also exhibited an increase in 4-hydroxynonenal, the expression of protein phosphatase 1 nuclear targeting subunit (PNUTS), p53 and caspase-3, and a decrease in protein phosphatase 1 (PP1) and MDM2 in the cochlea...
July 25, 2017: Neurobiology of Aging
https://www.readbyqxmd.com/read/28837644/prickle1-regulates-neurite-outgrowth-of-apical-spiral-ganglion-neurons-but-not-hair-cell-polarity-in-the-murine-cochlea
#14
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/28832600/hpn-07-a-free-radical-spin-trapping-agent-protects-against-functional-cellular-and-electrophysiological-changes-in-the-cochlea-induced-by-acute-acoustic-trauma
#15
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/28827351/mechanisms-of-synaptic-depression-at-the-hair-cell-ribbon-synapse-that-support-auditory-nerve-function
#16
Juan D Goutman
Inner hair cells (IHCs) in the cochlea are the mammalian phono-receptors, transducing sound energy into graded changes in membrane potentials, the so called "receptor potentials." Ribbon synapses between IHCs and auditory nerve neurons are responsible for converting receptor potentials into spike rates. The characteristics of auditory nerve responses to sound have been described extensively. For instance, persistent acoustic stimulation produces sensory adaptation, which is revealed as a reduction in neuronal spike rate with time constants in the range of milliseconds to seconds...
August 21, 2017: Proceedings of the National Academy of Sciences of the United States of America
https://www.readbyqxmd.com/read/28821654/wnt9a-can-influence-cell-fates-and-neural-connectivity-across-the-radial-axis-of-the-developing-cochlea
#17
Vidhya Munnamalai, Ulrike J Sienknecht, R Keith Duncan, M Katie Scott, Ankita Thawani, Kristen N Fantetti, Nadia M Atallah, Deborah J Biesemeier, Kuhn H Song, Kirsten Luethy, Eric Traub, Donna M Fekete
Vertebrate hearing organs manifest cellular asymmetries across the radial axis that underlie afferent versus efferent circuits between the inner ear and the brain. Thus, understanding the molecular control of patterning across this axis has important functional implications. Radial axis patterning begins before the cells become postmitotic, and is likely linked to the onset of asymmetric expression of secreted factors adjacent to the sensory primordium. This study explores one such asymmetrically-expressed gene, Wnt9a, that becomes restricted to the neural edge of the avian auditory organ, the basilar papilla, by embryonic day (E) 5...
August 14, 2017: Journal of Neuroscience: the Official Journal of the Society for Neuroscience
https://www.readbyqxmd.com/read/28818524/loss-of-sestrin-2-potentiates-the-early-onset-of-age-related-sensory-cell-degeneration-in-the-cochlea
#18
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
https://www.readbyqxmd.com/read/28818455/adipose-derived-stromal-cells-enhance-auditory-neuron-survival-in-an-animal-model-of-sensory-hearing-loss
#19
Philipp Schendzielorz, Maike Vollmer, Kristen Rak, Armin Wiegner, Nashwa Nada, Katrin Radeloff, Rudolf Hagen, Andreas Radeloff
BACKGROUND: A cochlear implant (CI) is an electronic prosthesis that can partially restore speech perception capabilities. Optimum information transfer from the cochlea to the central auditory system requires a proper functioning auditory nerve (AN) that is electrically stimulated by the device. In deafness, the lack of neurotrophic support, normally provided by the sensory cells of the inner ear, however, leads to gradual degeneration of auditory neurons with undesirable consequences for CI performance...
August 14, 2017: Cytotherapy
https://www.readbyqxmd.com/read/28814279/musashi-1-is-the-candidate-of-the-regulator-of-hair-cell-progenitors-during-inner-ear-regeneration
#20
Takahiro Wakasaki, Hiroaki Niiro, Siamak Jabbarzadeh-Tabrizi, Mitsuru Ohashi, Takashi Kimitsuki, Takashi Nakagawa, Shizuo Komune, Koichi Akashi
BACKGROUND: Hair cell loss in the cochlea is caused by ototoxic drugs, aging, and environmental stresses and could potentially lead to devastating pathophysiological effects. In adult mammals, hair cell loss is irreversible and may result in hearing and balance deficits. In contrast, nonmammalian vertebrates, including birds, can regenerate hair cells through differentiation of supporting cells and restore inner ear function, suggesting that hair cell progenitors are present in the population of supporting cells...
August 16, 2017: BMC Neuroscience
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