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cochlear hair cell

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https://www.readbyqxmd.com/read/29143984/crispr-cas9-mediated-knockout-of-lim-domain-only-4-retards-organ-of-corti-cell-growth
#1
Rajamani Rathinam, Rita Rosati, Samson Jamesdaniel
Lim-domain only 4 (LMO4) plays a critical role in mediating the ototoxic side-effects of cisplatin, a highly effective anti-cancer drug. However, the signaling mechanism by which cochlear LMO4 mediates otopathology is yet to be fully understood. Knockout cell culture models are useful tools for investigating the functional roles of novel genes and delineating associated signaling pathways. Therefore, LMO4 knockout organ of Corti cells were generated by using the CRISPR (clustered regularly interspersed short palindromic repeats)/Cas9 (CRISPR-associated protein 9) system...
November 16, 2017: Journal of Cellular Biochemistry
https://www.readbyqxmd.com/read/29139542/temporal-and-spatial-expression-patterns-of-hedgehog-receptors-in-the-developing-inner-and-middle-ear
#2
Jeong-Oh Shin, Harinarayana Ankamreddy, Naga Mahesh Jakka, Seokwon Lee, Un-Kyung Kim, Jinwoong Bok
The mammalian inner ear is a complex organ responsible for balance and hearing. Sonic hedgehog (Shh), a member of the Hedgehog (Hh) family of secreted proteins, has been shown to play important roles in several aspects of inner ear development, including dorsoventral axial specification, cochlear elongation, tonotopic patterning, and hair cell differentiation. Hh proteins initiate a downstream signaling cascade by binding to the Patched 1 (Ptch1) receptor. Recent studies have revealed that other types of co-receptors can also mediate Hh signaling, including growth arrest-specific 1 (Gas1), cell-adhesion molecules-related/down-regulated by oncogenes (Cdon), and biregional Cdon binding protein (Boc)...
2017: International Journal of Developmental Biology
https://www.readbyqxmd.com/read/29130968/immunohistochemical-localization-of-calbindin-d28k-parvalbumin-and-calretinin-in-the-superior-olivary-complex-of-circling-mice
#3
Jin-Koo Lee, Dhiraj Maskey, Myeung Ju Kim
The circling mouse serves as a hearing loss model. It has spontaneous tmie gene mutations that cause hair cell and cochlear degeneration. However, little is known about the role of the tmie gene in superior olivary complex (SOC) regions, in which sound information from the two ears is integrated and primarily relayed to the nuclei of the lateral lemniscus and inferior colliculus. Several studies have reported that abnormal calcium (Ca2+) homeostasis is associated with the pathology of hearing loss. This study investigated the distribution of Ca2+-binding proteins (CaBPs), such as calbindin D28k, parvalbumin, and calretinin, in the SOC of the circling mouse on postnatal day 16...
November 4, 2017: Cells, Tissues, Organs
https://www.readbyqxmd.com/read/29130354/delivery-of-adeno-associated-viral-vectors-in-adult-mammalian-inner-ear-cell-subtypes-without-auditory-dysfunction
#4
Yong Tao, Mingqian Huang, Yilai Shu, Adam Ruprecht, Hongyang Wang, Yong Tang, Luk H Vandenberghe, Qiuju Wang, Guangping Gao, Wei-Jia Kong, Zheng-Yi Chen
Hearing loss, including genetic hearing loss, is one of the most common forms of sensory deficits in human with limited options of treatment. Adeno-associated virus (AAV)-mediated gene transfer has been shown to effectively recover auditory functions in mouse models of genetic deafness when delivered at neonatal stages. However, the mouse cochlea is still developing at those time points whereas in human the newborn inner ears are already fully mature. For effective gene therapy to treat genetic deafness, it is necessary to determine whether or not AAV-mediated therapy can be equally effective in the fully mature mouse inner ear without causing damages to the inner ear...
November 12, 2017: Human Gene Therapy
https://www.readbyqxmd.com/read/29123468/a-model-based-approach-for-separating-the-cochlear-microphonic-from-the-auditory-nerve-neurophonic-in-the-ongoing-response-using-electrocochleography
#5
Tatyana E Fontenot, Christopher K Giardina, Douglas C Fitzpatrick
Electrocochleography (ECochG) is a potential clinically valuable technique for predicting speech perception outcomes in cochlear implant (CI) recipients, among other uses. Current analysis is limited by an inability to quantify hair cell and neural contributions which are mixed in the ongoing part of the response to low frequency tones. Here, we used a model based on source properties to account for recorded waveform shapes and to separate the combined signal into its components. The model for the cochlear microphonic (CM) was a sinusoid with parameters for independent saturation of the peaks and the troughs of the responses...
2017: Frontiers in Neuroscience
https://www.readbyqxmd.com/read/29122423/efferent-modulation-of-pre-neural-and-neural-distortion-products
#6
S B Smith, K Ichiba, D S Velenovsky, B Cone
Distortion product otoacoustic emissions (DPOAEs) and distortion product frequency following responses (DPFFRs) are respectively pre-neural and neural measurements associated with cochlear nonlinearity. Because cochlear nonlinearity is putatively linked to outer hair cell electromotility, DPOAEs and DPFFRs may provide complementary measurements of the human medial olivocochlear (MOC) reflex, which directly modulates outer hair cell function. In this study, we first quantified MOC reflex-induced DPOAE inhibition at spectral fine structure peaks in 22 young human adults with normal hearing...
October 26, 2017: Hearing Research
https://www.readbyqxmd.com/read/29118709/piccolo-promotes-vesicle-replenishment-at-a-fast-central-auditory-synapse
#7
Tanvi Butola, Carolin Wichmann, Tobias Moser
Piccolo and Bassoon are the two largest cytomatrix of the active zone (CAZ) proteins involved in scaffolding and regulating neurotransmitter release at presynaptic active zones (AZs), but have long been discussed as being functionally redundant. We employed genetic manipulation to bring forth and segregate the role of Piccolo from that of Bassoon at central auditory synapses of the cochlear nucleus-the endbulbs of Held. These synapses specialize in high frequency synaptic transmission, ideally poised to reveal even subtle deficits in the regulation of neurotransmitter release upon molecular perturbation...
2017: Frontiers in Synaptic Neuroscience
https://www.readbyqxmd.com/read/29116042/correction-the-murine-catecholamine-methyltransferase-mtomt-is-essential-for-mechanotransduction-by-cochlear-hair-cells
#8
Christopher L Cunningham, Zizhen Wu, Aria Jafari, Bo Zhao, Kat Schrode, Sarah Harkins-Perry, Amanda Lauer, Ulrich Müller
No abstract text is available yet for this article.
November 8, 2017: ELife
https://www.readbyqxmd.com/read/29114207/histone-deacetylase-inhibitors-are-protective-in-acute-but-not-in-chronic-models-of-ototoxicity
#9
Chao-Hui Yang, Zhiqi Liu, Deanna Dong, Jochen Schacht, Dev Arya, Su-Hua Sha
Previous studies have reported that modification of histones alters aminoglycoside-induced hair cell death and hearing loss. In this study, we investigated three FDA-approved histone deacetylase (HDAC) inhibitors (vorinostat/SAHA, belinostat, and panobinostat) as protectants against aminoglycoside-induced ototoxicity in murine cochlear explants and in vivo in both guinea pigs and CBA/J mice. Individually, all three HDAC inhibitors reduced gentamicin (GM)-induced hair cell loss in a dose-dependent fashion in explants...
2017: Frontiers in Cellular Neuroscience
https://www.readbyqxmd.com/read/29107847/the-fbn-rat-model-of-aging-investigation-of-abr-waveforms-and-ribbon-synapse-changes
#10
Rui Cai, Scott C Montgomery, Kaley A Graves, Donald M Caspary, Brandon C Cox
Age-related hearing loss is experienced by one-third of individuals aged 65 years and older and can be socially debilitating. Historically, there has been poor correlation between age-related threshold changes, loss of speech understanding, and loss of cochlear hair cells. We examined changes in ribbon synapse number at four different ages in Fisher Brown Norway rats, an extensively studied rat model of aging. In contrast to previous work in mice/Wistar rats, we found minimal ribbon synapse loss before 20 months, with significant differences in 24- and 28-month-old rats at 4 kHz...
October 9, 2017: Neurobiology of Aging
https://www.readbyqxmd.com/read/29107558/esrp1-mutations-cause-hearing-loss-due-to-defects-in-alternative-splicing-that-disrupt-cochlear-development
#11
Alex M Rohacek, Thomas W Bebee, Richard K Tilton, Caleb M Radens, Chris McDermott-Roe, Natoya Peart, Maninder Kaur, Michael Zaykaner, Benjamin Cieply, Kiran Musunuru, Yoseph Barash, John A Germiller, Ian D Krantz, Russ P Carstens, Douglas J Epstein
Alternative splicing contributes to gene expression dynamics in many tissues, yet its role in auditory development remains unclear. We performed whole-exome sequencing in individuals with sensorineural hearing loss (SNHL) and identified pathogenic mutations in Epithelial Splicing-Regulatory Protein 1 (ESRP1). Patient-derived induced pluripotent stem cells showed alternative splicing defects that were restored upon repair of an ESRP1 mutant allele. To determine how ESRP1 mutations cause hearing loss, we evaluated Esrp1(-/-) mouse embryos and uncovered alterations in cochlear morphogenesis, auditory hair cell differentiation, and cell fate specification...
November 6, 2017: Developmental Cell
https://www.readbyqxmd.com/read/29093662/tmc2-modifies-permeation-properties-of-the-mechanoelectrical-transducer-channel-in-early-postnatal-mouse-cochlear-outer-hair-cells
#12
Laura F Corns, Jing-Yi Jeng, Guy P Richardson, Corné J Kros, Walter Marcotti
The ability of cochlear hair cells to convert sound into receptor potentials relies on the mechanoelectrical transducer (MET) channels present in their stereociliary bundles. There is strong evidence implying that transmembrane channel-like protein (TMC) 1 contributes to the pore-forming subunit of the mature MET channel, yet its expression is delayed (~>P5 in apical outer hair cells, OHCs) compared to the onset of mechanotransduction (~P1). Instead, the temporal expression of TMC2 coincides with this onset, indicating that it could be part of the immature MET channel...
2017: Frontiers in Molecular Neuroscience
https://www.readbyqxmd.com/read/29092611/modeling-signal-propagation-in-the-human-cochlea
#13
Stephen T Neely, Daniel M Rasetshwane
The level-dependent component of the latency of human auditory brainstem responses (ABR) to tonebursts decreases by about 38% for every 20-dB increase in stimulus level over a wide range of both frequency and level [Neely, Norton, Gorga, and Jesteadt (1998). J. Acoust. Soc. Am. 31, 87-97]. This level-dependence has now been simulated in an active, nonlinear, transmission-line model of cochlear mechanics combined with an adaptation stage. The micromechanics in this model are similar to previous models except that a dual role is proposed for the tectorial membrane (TM): (1) passive sharpening the tuning of sensory-cell inputs (relative to basilar-membrane vibrations) and (2) providing an optimal phase shift (relative to basilar-membrane vibrations) of outer-hair-cell feedback forces, so that amplification is restricted to a limited range of frequencies...
October 2017: Journal of the Acoustical Society of America
https://www.readbyqxmd.com/read/29084395/electrophysiological-evidence-of-the-basilar-membrane-travelling-wave-and-frequency-place-coding-of-sound-in-cochlear-implant-recipients
#14
Luke Campbell, Christofer Bester, Claire Iseli, David Sly, Adrian Dragovic, Anthony W Gummer, Stephen O'Leary
AIM: To obtain direct evidence for the cochlear travelling wave in humans by performing electrocochleography from within the cochlea in subjects implanted with an auditory prosthesis. BACKGROUND: Sound induces a travelling wave that propagates along the basilar membrane, exhibiting cochleotopic tuning with a frequency-dependent phase delay. To date, evoked potentials and psychophysical experiments have supported the presence of the travelling wave in humans, but direct measurements have not been made...
2017: Audiology & Neuro-otology
https://www.readbyqxmd.com/read/29084289/neuronal-differentiation-of-hair-follicle-bulge-derived-stem-cells-co-cultured-with-mouse-cochlear-modiolus-explants
#15
Timo Schomann, Laura Mezzanotte, John C M J De Groot, Marcelo N Rivolta, Sanne H Hendriks, Johan H M Frijns, Margriet A Huisman
Stem-cell-based repair of auditory neurons may represent an attractive therapeutic option to restore sensorineural hearing loss. Hair-follicle-bulge-derived stem cells (HFBSCs) are promising candidates for this type of therapy, because they (1) have migratory properties, enabling migration after transplantation, (2) can differentiate into sensory neurons and glial cells, and (3) can easily be harvested in relatively high numbers. However, HFBSCs have never been used for this purpose. We hypothesized that HFBSCs can be used for cell-based repair of the auditory nerve and we have examined their migration and incorporation into cochlear modiolus explants and their subsequent differentiation...
2017: PloS One
https://www.readbyqxmd.com/read/29081120/-role-of-green-tea-polyphenols-in-noise-induced-hearing-loss
#16
Y H Li, P S Jiang, Y W Su, Y M Liu
Objective: To investigate the role and mechanism of action of green tea polyphenols in noise-induced hearing loss. Methods: Male specific pathogen-free guinea pigs were randomly divided into normal control group with 9 guinea pigs, noise exposure group with 36 guinea pigs, and green tea polyphenol intervention group with 36 guinea pigs. Auditory brainstem response (ABR) threshold shift was examined before noise exposure and at 1, 3, 7, and 14 days of noise exposure. The surface preparation of cochlear basilar membrane was used for hair cell count and the morphology of hair cells was also observed...
August 20, 2017: Chinese Journal of Industrial Hygiene and Occupational Diseases
https://www.readbyqxmd.com/read/29069081/spatiotemporal-changes-in-the-distribution-of-lhfpl5-in-mice-cochlear-hair-bundles-during-development-and-in-the-absence-of-pcdh15
#17
Shanthini Mahendrasingam, Robert Fettiplace, Kumar N Alagramam, Ellen Cross, David N Furness
Mechanosensory transduction by vertebrate hair cells depends on a protein complex at the tips of shorter stereocilia associated with mechanoelectrical transduction channels activated by tip links in the hair bundle. In mammalian hair cells, this complex includes transmembrane channel-like protein subunit 1 (TMC1), lipoma HMGIC fusion partner-like 5 protein (LHFPL5) and protocadherin 15 (PCDH15), a lower-end component of the tip link. TMC1 interacts with LHFPL5 and PCDH15 but how the complex develops to maturity, and the relationships between these proteins, remains uncertain...
2017: PloS One
https://www.readbyqxmd.com/read/29068588/potential-protective-effect-of-n-acetyl-cysteine-in-acoustic-trauma-an-experimental-study-using-scanning-electron-microscopy
#18
Servet Ada, Deniz Hanci, Seçkin Ulusoy, Djanan Vejselova, Dilek Burukoglu, Nuray Bayar Muluk, Cemal Cingi
BACKGROUND: Oxidative stress has been associated with pathological processes involved in acoustic trauma. OBJECTIVES: In this prospective experimental study, we investigated the potential preventive effect of N-acetyl cysteine (NAC) in rats exposed to acoustic trauma (AT). Light microscopic and scanning electron microscopic (SEM) evaluations were performed. MATERIAL AND METHODS: Healthy Wistar albino rats (n = 18) were divided into 3 groups: group 1 (control group, n = 6), group 2 (acoustic trauma group, n = 6), and group 3 (AT+NAC group, n = 6)...
September 2017: Advances in Clinical and Experimental Medicine: Official Organ Wroclaw Medical University
https://www.readbyqxmd.com/read/29059194/role-of-neuropilin-1-semaphorin-3a-signaling-in-the-functional-and-morphological-integrity-of-the-cochlea
#19
Pezhman Salehi, Marshall X Ge, Usha Gundimeda, Leah Michelle Baum, Homero Lael Cantu, Joel Lavinsky, Litao Tao, Anthony Myint, Charlene Cruz, Juemei Wang, Angeliki Maria Nikolakopoulou, Carolina Abdala, Matthew William Kelley, Takahiro Ohyama, Thomas Matthew Coate, Rick A Friedman
Neuropilin-1 (Nrp1) encodes the transmembrane cellular receptor neuropilin-1, which is associated with cardiovascular and neuronal development and was within the peak SNP interval on chromosome 8 in our prior GWAS study on age-related hearing loss (ARHL) in mice. In this study, we generated and characterized an inner ear-specific Nrp1 conditional knockout (CKO) mouse line because Nrp1 constitutive knockouts are embryonic lethal. In situ hybridization demonstrated weak Nrp1 mRNA expression late in embryonic cochlear development, but increased expression in early postnatal stages when cochlear hair cell innervation patterns have been shown to mature...
October 2017: PLoS Genetics
https://www.readbyqxmd.com/read/29056431/effects-of-surgical-lesions-on-choline-acetyltransferase-activity-in-the-cat-cochlea
#20
Mark J Frilling, Gregory J Wiet, Donald A Godfrey, Judy A Parli, Jon D Dunn, C David Ross
Although it is well established that the choline acetyltransferase (ChAT, the enzyme for acetylcholine synthesis) in the mammalian cochlea is associated with its olivocochlear innervation, the distribution of this innervation in the cochlea varies somewhat among mammalian species. The quantitative distribution of ChAT activity in the cochlea has been reported for guinea pigs and rats. The present study reports the distribution of ChAT activity within the organ of Corti among the three turns of the cat cochlea and the effects of removing olivocochlear innervation either by a lateral cut aimed to totally transect the left olivocochlear bundle or a more medial cut additionally damaging the superior olivary complex on the same side...
October 10, 2017: Hearing Research
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