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https://www.readbyqxmd.com/read/30333726/loss-of-arhgef6-causes-hair-cell-stereocilia-deficits-and-hearing-loss-in-mice
#1
Chengwen Zhu, Cheng Cheng, Yanfei Wang, Waqas Muhammad, Shuang Liu, Weijie Zhu, Buwei Shao, Zhong Zhang, Xiaoqian Yan, Qingqing He, Zhengrong Xu, Chenjie Yu, Xiaoyun Qian, Ling Lu, Shasha Zhang, Yuan Zhang, Wei Xiong, Xia Gao, Zhigang Xu, Renjie Chai
ARHGEF6 belongs to the family of guanine nucleotide exchange factors (GEFs) for Rho GTPases, and it specifically activates Rho GTPases CDC42 and RAC1. Arhgef6 is the X-linked intellectual disability gene also known as XLID46, and clinical features of patients carrying Arhgef6 mutations include intellectual disability and, in some cases, sensorineural hearing loss. Rho GTPases act as molecular switches in many cellular processes. Their activities are regulated by binding or hydrolysis of GTP, which is facilitated by GEFs and GTPase-activating proteins, respectively...
2018: Frontiers in Molecular Neuroscience
https://www.readbyqxmd.com/read/30333133/regulation-of-actin-based-apical-structures-on-epithelial-cells
#2
REVIEW
Thaher Pelaseyed, Anthony Bretscher
Cells of transporting epithelia are characterized by the presence of abundant F-actin-based microvilli on their apical surfaces. Likewise, auditory hair cells have highly reproducible rows of apical stereocilia (giant microvilli) that convert mechanical sound into an electrical signal. Analysis of mutations in deaf patients has highlighted the critical components of tip links between stereocilia, and related structures that contribute to the organization of microvilli on epithelial cells have been found. Ezrin/radixin/moesin (ERM) proteins, which are activated by phosphorylation, provide a critical link between the plasma membrane and underlying actin cytoskeleton in surface structures...
October 17, 2018: Journal of Cell Science
https://www.readbyqxmd.com/read/30319226/cochlear-proteins-associated-with-noise-induced-hearing-loss-an-update
#3
REVIEW
Ruchika K Jain, Shubhangi K Pingle, Rajani G Tumane, Lucky R Thakkar, Aruna A Jawade, Anand Barapatre, Minal Trivedi
Noise-induced hearing loss (NIHL) is one of the major occupational disease that has influence on the quality of life of mining workers. Several reports suggest NIHL is attributed to noise exposure at workplace and approximately 16% of hearing loss is due to it. NIHL occurs as a result of exposure to high-level noise (>85 dB) in the workplace. Noise disrupts proteins present in the micromachinery of the ear that is required for mechano-electric transduction of sound waves. High-level noise exposure can lead to hearing impairment owing to mechanical and metabolic exhaustion in cochlea, the major organ responsible for resilience of sound...
May 2018: Indian Journal of Occupational and Environmental Medicine
https://www.readbyqxmd.com/read/30291150/function-and-dysfunction-of-tmc-channels-in-inner-ear-hair-cells
#4
David P Corey, Nurunisa Akyuz, Jeffrey R Holt
The TMC1 channel was identified as a protein essential for hearing in mouse and human, and recognized as one of a family of eight such proteins in mammals. The TMC family is part of a superfamily of seven branches, which includes the TMEM16s. Vertebrate hair cells express both TMC1 and TMC2. They are located at the tips of stereocilia and are required for hair cell mechanotransduction. TMC1 assembles as a dimer and its similarity to the TMEM16s has enabled a predicted tertiary structure with an ion conduction pore in each subunit of the dimer...
October 5, 2018: Cold Spring Harbor Perspectives in Medicine
https://www.readbyqxmd.com/read/30280293/ripor2-is-involved-in-auditory-hair-cell-stereociliary-bundle-structure-and-orientation
#5
Oscar Diaz-Horta, Clemer Abad, Filiz Basak Cengiz, Guney Bademci, Pat Blackwelder, Katherina Walz, Mustafa Tekin
RIPOR2 (previously known as FAM65B) localizes to stereocilia of auditory hair cells and causes deafness when its function is disturbed by mutations. Here, we demonstrate that during the morphogenesis of the hair cell bundle, absence of Ripor2 affects the orientation of this key subcellular structure. We show that Ripor2 interacts with Myh9, a protein encoded by a known deafness gene. Absence of Ripor2 is associated with low Myh9 abundance in the mouse cochlea despite increased amount of Myh9 transcripts. While Myh9 is mainly expressed in stereocilia, a phosphorylated form of Myh9 is particularly enriched in the kinocilium...
October 3, 2018: Journal of Molecular Medicine: Official Organ of the "Gesellschaft Deutscher Naturforscher und Ärzte"
https://www.readbyqxmd.com/read/30268784/protective-effect-of-n-acetylcysteine-against-cisplatin-ototoxicity-in-rats-a-study-with-hearing-tests-and-scanning-electron-microscopy
#6
Mehmet Akif Somdaş, İnayet Güntürk, Esra Balcıoğlu, Deniz Avcı, Cevat Yazıcı, Saim Özdamar
INTRODUCTION: Ototoxicity is a health problem appearing after powerful treatments in serious health conditions. It is sometimes inevitable when treatment of the serious disease is required. Cisplatin is an antineoplastic agent which was investigated previously to reveal increased nitrogen and reactive oxygen radicals that damages hair cells, resulting in ototoxicity. N-acetylcysteine, previously shown to decrease ototoxicity caused by different agents, is known to be a powerful in vitro antioxidant...
September 14, 2018: Brazilian Journal of Otorhinolaryngology
https://www.readbyqxmd.com/read/30254566/deletion-of-kncn-does-not-affect-kinocilium-and-stereocilia-bundle-morphogenesis-and-mechanotransduction-in-cochlear-hair-cells
#7
Qun Hu, Li Guo, Jie Li, Chenmeng Song, Lisheng Yu, David Z Z He, Wei Xiong
Auditory hair cells possess stunning cilia structure that composes of a bundle of stereocilia for mechano-electrical transduction and a single kinocilium for guiding the polarity of hair bundle towards maturation. However, the molecules underlying kinocilium function have not yet been fully understood. Hence, the proteins involved in hair bundle development and function are of a large interest. From a fine microarray analysis, we found that kinocilin ( Kncn ) was enriched in hair cell specific expression profile...
2018: Frontiers in Molecular Neuroscience
https://www.readbyqxmd.com/read/30242501/knockout-of-ush2a-gene-in-zebrafish-causes-hearing-impairment-and-late-onset-rod-cone-dystrophy
#8
Shanshan Han, Xiliang Liu, Shanglun Xie, Meng Gao, Fei Liu, Shanshan Yu, Peng Sun, Changquan Wang, Stephen Archacki, Zhaojing Lu, Xuebin Hu, Yayun Qin, Zhen Qu, Yuwen Huang, Yuexia Lv, Jiayi Tu, Jingzhen Li, Tinsae Assefa Yimer, Tao Jiang, Zhaohui Tang, Daji Luo, Fangyi Chen, Mugen Liu
Most cases of Usher syndrome type II (USH2) are due to mutations in the USH2A gene. There are no effective treatments or ideal animal models for this disease, and the pathological mechanisms of USH2 caused by USH2A mutations are still unknown. Here, we constructed a ush2a knockout (ush2a-/- ) zebrafish model using TALEN technology to investigate the molecular pathology of USH2. An early onset auditory disorder and abnormal morphology of inner ear stereocilia were identified in the ush2a-/- zebrafish. Consequently, the disruption of Ush2a in zebrafish led to a hearing impairment, like that in mammals...
September 21, 2018: Human Genetics
https://www.readbyqxmd.com/read/30238654/gene-expression-and-immunolocalization-of-low-affinity-neurotrophin-receptor-p75-in-rabbit-male-reproductive-tract-during-sexual-maturation
#9
Ana Sanchez-Rodriguez, Maria Arias-Alvarez, Pilar G Rebollar, Jose M Bautista, Pedro L Lorenzo, Rosa M Garcia-Garcia
This study reports the gene expression and immunolocalization of the low-affinity neurotrophin receptor, p75, in accessory glands (prostate and bulbourethral glands) and epididymis (caput and cauda) of male rabbits during sexual maturation. We showed that p75 was expressed in all tissues studied with similar mRNA levels during this period. However, it was differentially immunolocalized in bulbourethral glands and stereocilia of epididymis. These findings may be related with some remodeling processes in the accessory glands during sexual maturation, which could be associated with sperm maturation...
September 2018: Reproduction in Domestic Animals, Zuchthygiene
https://www.readbyqxmd.com/read/30196827/lower-level-noise-exposure-that-produces-only-tts-modulates-the-immune-homeostasis-of-cochlear-macrophages
#10
Mitchell D Frye, Celia Zhang, Bo Hua Hu
Noise exposure producing temporary threshold shifts (TTS) has been demonstrated to cause permanent changes to cochlear physiology and hearing function. Several explanations have been purported to underlie these long-term changes in cochlear function, such as damage to sensory cell stereocilia and synaptic connections between sensory cells and their innervation by spiral ganglion neurons, and demyelination of the auditory nerve. Though these structural defects have been implicated in hearing difficulty, cochlear responses to this stress damage remains poorly understood...
October 15, 2018: Journal of Neuroimmunology
https://www.readbyqxmd.com/read/30179640/nonlinear-calcium-ion-waves-along-actin-filaments-control-active-hair-bundle-motility
#11
Jack A Tuszynski, Miljko V Sataric, Dalibor L Sekulic, Bogdan M Sataric, Slobodan Zdravkovic
Calcium ions (Ca2+ ) tune and control numerous diverse aspects of cochlear and vestibular physiological processes. This paper is focused on the Ca2+ control of mechanotransduction in sensory hair cells in the context of polyelectrolyte properties of actin filaments within the hair-bundles of inner ear. These actin filaments appear to serve as efficient pathways for the flow of Ca2+ ions inside stereocilia. We showed how this can be utilized for tuning of force-generating myosin motors. In an established model, we unified the Ca2+ nonlinear dynamics involved in the control of myosin adaptation motors with mechanical displacements of hair-bundles...
September 1, 2018: Bio Systems
https://www.readbyqxmd.com/read/30159668/tprn-is-essential-for-the-integrity-of-stereociliary-rootlet-in-cochlear-hair-cells-in-mice
#12
Yuqin Men, Xiujuan Li, Hailong Tu, Aizhen Zhang, Xiaolong Fu, Zhishuo Wang, Yecheng Jin, Congzhe Hou, Tingting Zhang, Sen Zhang, Yichen Zhou, Boqin Li, Jianfeng Li, Xiaoyang Sun, Haibo Wang, Jiangang Gao
Tprn encodes the taperin protein, which is concentrated in the tapered region of hair cell stereocilia in the inner ear. In humans, TPRN mutations cause autosomal recessive nonsyndromic deafness (DFNB79) by an unknown mechanism. To determine the role of Tprn in hearing, we generated Tprn-null mice by clustered regularly interspaced short palindromic repeat/Cas9 genome-editing technology from a CBA/CaJ background. We observed significant hearing loss and progressive degeneration of stereocilia in the outer hair cells of Tprn-null mice starting from postnatal day 30...
August 30, 2018: Frontiers of Medicine
https://www.readbyqxmd.com/read/30135646/evolutionary-and-developmental-biology-provide-insights-into-the-regeneration-of-organ-of-corti-hair-cells
#13
REVIEW
Karen L Elliott, Bernd Fritzsch, Jeremy S Duncan
We review the evolution and development of organ of Corti hair cells with a focus on their molecular differences from vestibular hair cells. Such information is needed to therapeutically guide organ of Corti hair cell development in flat epithelia and generate the correct arrangement of different hair cell types, orientation of stereocilia, and the delayed loss of the kinocilium that are all essential for hearing, while avoiding driving hair cells toward a vestibular fate. Highlighting the differences from vestibular organs and defining what is known about the regulation of these differences will help focus future research directions toward successful restoration of an organ of Corti following long-term hair cell loss...
2018: Frontiers in Cellular Neuroscience
https://www.readbyqxmd.com/read/30131691/-cdh23-methylation-status-and-presbycusis-risk-in-elderly-women
#14
Amal Bouzid, Ibtihel Smeti, Amine Chakroun, Salma Loukil, Abdullah Ahmed Gibriel, Mhamed Grati, Abdelmonem Ghorbel, Saber Masmoudi
Introduction : Presbycusis, an age-related hearing impairment (ARHI) disease, is the most common cause for HI in adults worldwide. One of the best candidate genes for ARHI susceptibility is Cadherin 23 ( CDH23 ) which encodes stereocilia tip-links of the inner ear sensory hair cell. Although alterations in the methylation status of CpG dinucleotides across various genes were reported to be associated with HI, methylation changes in CDH23 gene have not been reported previously. Objectives : This study aimed at investigating whether DNA methylation level of CDH23 gene at intragenic CpG island overlapping an exonic-intronic region at position chr10:73565570-73565827 (GRCh37/hg19) could be risk factor associated with ARHI...
2018: Frontiers in Aging Neuroscience
https://www.readbyqxmd.com/read/30123247/knock-in-mice-with-myo3a-y137c-mutation-displayed-progressive-hearing-loss-and-hair-cell-degeneration-in-the-inner-ear
#15
Peipei Li, Zongzhuang Wen, Guangkai Zhang, Aizhen Zhang, Xiaolong Fu, Jiangang Gao
Myo3a is expressed in cochlear hair cells and retinal cells and is responsible for human recessive hereditary nonsyndromic deafness (DFNB30). To investigate the mechanism of DFNB30-type deafness, we established a mouse model of Myo3a kinase domain Y137C mutation by using CRISPR/Cas9 system. No difference in hearing between 2-month-old Myo3a mutant mice and wild-type mice was observed. The hearing threshold of the ≥6-month-old mutant mice was significantly elevated compared with that of the wild-type mice...
2018: Neural Plasticity
https://www.readbyqxmd.com/read/30108230/tmc2-expression-partially-restores-auditory-function-in-a-mouse-model-of-dfnb7-b11-deafness-caused-by-loss-of-tmc1-function
#16
Hiroshi Nakanishi, Kiyoto Kurima, Bifeng Pan, Philine Wangemann, Tracy S Fitzgerald, Gwenaëlle S Géléoc, Jeffrey R Holt, Andrew J Griffith
Mouse Tmc1 and Tmc2 are required for sensory transduction in cochlear and vestibular hair cells. Homozygous Tmc1∆/∆ mice are deaf, Tmc2∆/∆ mice have normal hearing, and double homozygous Tmc1∆/∆ ; Tmc2∆/∆ mice have deafness and profound vestibular dysfunction. These phenotypes are consistent with their different spatiotemporal expression patterns. Tmc1 expression is persistent in cochlear and vestibular hair cells, whereas Tmc2 expression is transient in cochlear hair cells but persistent in vestibular hair cells...
August 14, 2018: Scientific Reports
https://www.readbyqxmd.com/read/30082452/molecular-structure-of-the-hair-cell-mechanoelectrical-transduction-complex
#17
Christopher L Cunningham, Ulrich Müller
Cochlear hair cells employ mechanically gated ion channels located in stereocilia that open in response to sound wave-induced motion of the basilar membrane, converting mechanical stimulation to graded changes in hair cell membrane potential. Membrane potential changes in hair cells cause neurotransmitter release from hair cells that initiate electrical signals in the nerve terminals of afferent fibers from spiral ganglion neurons. These signals are then propagated within the central nervous system (CNS) to mediate the sensation of hearing...
August 6, 2018: Cold Spring Harbor Perspectives in Medicine
https://www.readbyqxmd.com/read/30082328/null-mutation-of-the-fascin2-gene-by-talen-leading-to-progressive-hearing-loss-and-retinal-degeneration-in-c57bl-6j-mice
#18
Xiang Liu, Mengmeng Zhao, Yi Xie, Ping Li, Oumei Wang, Bingxin Zhou, Linlin Yang, Yao Nie, Lin Cheng, Xicheng Song, Changzhu Jin, Fengchan Han
Fascin2 (FSCN2) is an actin cross-linking protein that is mainly localized in retinas and in the stereocilia of hair cells. Earlier studies showed that a deletion mutation in human FASCIN2 ( FSCN2 ) gene could cause autosomal dominant retinitis pigmentosa. Recent studies have indicated that a missense mutation in mouse Fscn2 gene (R109H) can contribute to the early onset of hearing loss in DBA/2J mice. To explore the function of the gene, Fscn2 was knocked out using TALEN (transcription activator-like effector nucleases) on the C57BL/6J background...
October 3, 2018: G3: Genes—Genomes—Genetics
https://www.readbyqxmd.com/read/30061390/fgfr1-mediated-protocadherin-15-loading-mediates-cargo-specificity-during-intraflagellar-transport-in-inner-ear-hair-cell-kinocilia
#19
Akira Honda, Tomoko Kita, Shri Vidhya Seshadri, Kazuyo Misaki, Zamal Ahmed, John E Ladbury, Guy P Richardson, Shigenobu Yonemura, Raj K Ladher
The mechanosensory hair cells of the inner ear are required for hearing and balance and have a distinctive apical structure, the hair bundle, that converts mechanical stimuli into electrical signals. This structure comprises a single cilium, the kinocilium, lying adjacent to an ensemble of actin-based projections known as stereocilia. Hair bundle polarity depends on kinociliary protocadherin-15 (Pcdh15) localization. Protocadherin-15 is found only in hair-cell kinocilia, and is not localized to the primary cilia of adjacent supporting cells...
August 14, 2018: Proceedings of the National Academy of Sciences of the United States of America
https://www.readbyqxmd.com/read/30055728/effect-of-sleep-deprivation-on-hearing-levels-in-rats
#20
Jae Hyun Jung, Miryeong Kim, Seung Jae Lee, Eunsang Lee, Se A Lee, Jong Dae Lee, Ji Ho Choi, Bo Gyung Kim
OBJECTIVES: To our knowledge, the influence of sleep deprivation on hearing levels has yet to be assessed in animals. Therefore, we evaluated whether auditory function was affected by sleep deprivation in rats. METHODS: Male Wistar rats (aged 9 weeks, weighing 300-400 g) were used for the study and were randomly assigned to a control (n = 15) or sleep deprivation group (n = 12). Hearing levels were evaluated at baseline and 9 days after sleep deprivation using auditory evoked brainstem responses (ABRs) and distortion product otoacoustic emission (DPOAE) measurements...
September 2018: International Journal of Pediatric Otorhinolaryngology
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