keyword
MENU ▼
Read by QxMD icon Read
search

spiral ganglion neuron

keyword
https://www.readbyqxmd.com/read/29665173/a-novel-gjb2-compound-heterozygous-mutation-c-257c-g-p-t86r-c-176del16-p-g59a-fs-18-causes-sensorineural-hearing-loss-in-a-chinese-family
#1
Xi Shi, Yan Zhang, Shiwei Qiu, Wei Zhuang, Na Yuan, Tiantian Sun, Jian Gao, Yuehua Qiao, Ke Liu
OBJECTIVE: To investigate whether a novel compound heterozygous mutations c.257C>G (p.T86R)/c.176del16 (p.G59A fs*18) in GJB2 result in hearing loss. METHODS: Allele-specific PCR-based universal array (ASPUA) screening and sequence analysis were applied to identify these mutations. 3D model was built to perform molecular dynamics (MD) simulation to verify the susceptibility of the mutations. Furthermore, WT- and Mut-GJB2 DNA fragments, containing the mutation of c...
April 17, 2018: Journal of Clinical Laboratory Analysis
https://www.readbyqxmd.com/read/29661613/functions-of-cabp1-and-cabp2-in-the-peripheral-auditory-system
#2
Tian Yang, Ning Hu, Tina Pangršič, Steven Green, Marlan Hansen, Amy Lee
CaBPs are a family of Ca2+ binding proteins related to calmodulin. Two CaBP family members, CaBP1 and CaBP2, are highly expressed in the cochlea. Here, we investigated the significance of CaBP1 and CaBP2 for hearing in mice lacking expression of these proteins (CaBP1 KO and CaBP2 KO) using auditory brain responses (ABRs) and distortion product otoacoustic emissions (DPOAEs). In CaBP1 KO mice, ABR wave I was larger in amplitude, and shorter in latency and faster in decay, suggestive of enhanced synchrony of auditory nerve fibers...
April 9, 2018: Hearing Research
https://www.readbyqxmd.com/read/29659185/the-cochlear-spiral-ganglion-neurons-the-auditory-portion-of-the-viii-nerve
#3
Francisco Carricondo, Bárbara Romero-Gómez
The VIII nerve is formed by sensory neurons that innervate the inner ear, i.e. the vestibular and the auditory receptors. Neurons of the auditory portion, the cochlear afferent fibers that innervate the sensory hair cells of the organ of Corti, have their somas in the cochlear spiral ganglion where two types of neurons can be distinguished. Afferent type-I neurons are the 95% of the total population. Bipolar and myelinated fibers, each one innervates only one cochlear inner hair cell (IHC). In contrast, afferent type-II neurons are only the 5% of the spiral ganglion population...
April 16, 2018: Anatomical Record: Advances in Integrative Anatomy and Evolutionary Biology
https://www.readbyqxmd.com/read/29649050/temporal-bone-histopathology-in-cockayne-syndrome
#4
Ophir Handzel, Joseph B Nadol
: Cockayne syndrome (CS) is a rare autosomal recessive syndrome resulting in defective DNA repair. Its features include cachectic dwarfism, hearing loss, skin hypersensitivity to sunlight, premature aging, and dementia. Presented is a right temporal bone of a patient who died at the age of 29 years. The clinical course was compatible with type 1 CS, the classical form. Homozygous missense variant in the ERCC6 gene (Excision Repair Cross-Complementation group 6) was found, compatible with CS complementation group B...
April 11, 2018: Otology & Neurotology
https://www.readbyqxmd.com/read/29593497/reprogramming-glia-into-neurons-in-the-peripheral-auditory-system-as-a-solution-for-sensorineural-hearing-loss-lessons-from-the-central-nervous-system
#5
REVIEW
Steven J Meas, Chun-Li Zhang, Alain Dabdoub
Disabling hearing loss affects over 5% of the world's population and impacts the lives of individuals from all age groups. Within the next three decades, the worldwide incidence of hearing impairment is expected to double. Since a leading cause of hearing loss is the degeneration of primary auditory neurons (PANs), the sensory neurons of the auditory system that receive input from mechanosensory hair cells in the cochlea, it may be possible to restore hearing by regenerating PANs. A direct reprogramming approach can be used to convert the resident spiral ganglion glial cells into induced neurons to restore hearing...
2018: Frontiers in Molecular Neuroscience
https://www.readbyqxmd.com/read/29590114/mutations-in-diphosphoinositol-pentakisphosphate-kinase-ppip5k2-are-associated-with-hearing-loss-in-human-and-mouse
#6
Rizwan Yousaf, Chunfang Gu, Zubair M Ahmed, Shaheen N Khan, Thomas B Friedman, Sheikh Riazuddin, Stephen B Shears, Saima Riazuddin
Autosomal recessive nonsyndromic hearing loss is a genetically heterogeneous disorder. Here, we report a severe-to-profound sensorineural hearing loss locus, DFNB100 on chromosome 5q13.2-q23.2. Exome enrichment followed by massive parallel sequencing revealed a c.2510G>A transition variant in PPIP5K2 that segregated with DFNB100-associated hearing loss in two large apparently unrelated Pakistani families. PPIP5Ks enzymes interconvert 5-IP7 and IP8, two key members of the inositol pyrophosphate (PP-IP) cell-signaling family...
March 28, 2018: PLoS Genetics
https://www.readbyqxmd.com/read/29587485/wnt-signaling-activates-tigar-and-protects-against-cisplatin-induced-spiral-ganglion-neuron-damage-in-the-mouse-cochlea
#7
Wenwen Liu, Xiaochen Xu, Zhaomin Fan, Gaoying Sun, Yuechen Han, Daogong Zhang, Lei Xu, Mingming Wang, Xue Wang, Shasha Zhang, Mingliang Tang, Jianfeng Li, Renjie Chai, Haibo Wang
AIMS: Cisplatin can damage spiral ganglion neurons (SGNs) and cause sensorineural hearing loss. Wnt activation protects against neomycin-induced hair cell damage in the mouse cochlea, but the role of Wnt signaling in protecting SGNs from cisplatin treatment has not yet been elucidated. This study was designed to investigate the neuroprotective effects of Wnt signaling against cisplatin-induced SGN damage. RESULTS: Firstly, we found that Wnt signaling was activated in SGNs after cisplatin treatment...
March 27, 2018: Antioxidants & Redox Signaling
https://www.readbyqxmd.com/read/29584754/long-term-delivery-of-brain-derived-neurotrophic-factor-bdnf-from-nanoporous-silica-nanoparticles-improves-the-survival-of-spiral-ganglion-neurons-in-vitro
#8
Nadeschda Schmidt, Jennifer Schulze, Dawid P Warwas, Nina Ehlert, Thomas Lenarz, Athanasia Warnecke, Peter Behrens
Sensorineural hearing loss (SNHL) can be overcome by electrical stimulation of spiral ganglion neurons (SGNs) via a cochlear implant (CI). Restricted CI performance results from the spatial gap between the SGNs and the electrode, but the efficacy of CI is also limited by the degeneration of SGNs as one consequence of SHNL. In the healthy cochlea, the survival of SGNs is assured by endogenous neurotrophic support. Several applications of exogenous neurotrophic supply have been shown to reduce SGN degeneration in vitro and in vivo...
2018: PloS One
https://www.readbyqxmd.com/read/29570699/microrna-194-regulates-the-development-and-differentiation-of-sensory-patches-and-statoacoustic-ganglion-of-inner-ear-by-fgf4
#9
Hui Cao, Jianbo Shi, Jintao Du, Kaitian Chen, Chang Dong, Di Jiang, Hongyan Jiang
BACKGROUND MicroRNA 194 is involved in the differentiation of various types of cells, such as adipose derived stem cells, human embryonic stem cells, and bone marrow mesenchymal stem cells. Previously, we found that miR-194 was highly expressed in the inner ear sensory patch and neurons in mice embryos. However, the role of miR-194 in the development of the inner ear and its underlying mechanism have not been elucidated yet. MATERIAL AND METHODS The expression level of miR-194 has been altered by using antisense morpholino oligonucleotides (MO) and synthesized miRNAs in zebrafish...
March 23, 2018: Medical Science Monitor: International Medical Journal of Experimental and Clinical Research
https://www.readbyqxmd.com/read/29570158/growth-hormone-promotes-neurite-growth-of-spiral-ganglion-neurons
#10
Jennis Gabrielpillai, Christin Geissler, Belinda Stock, Timo Stöver, Marc Diensthuber
Intact spiral ganglion neurons are a specific requirement for hearing rehabilitation in deaf patients by cochlear implantation. Neurotrophic growth factors have been proposed as effective tools to protect and regenerate spiral ganglion neurons that are degenerated in the majority of patients suffering from hearing loss. Here, we show that growth hormone (GH), a pleiotropic growth factor whose neurotrophic role in the inner ear is still unclear, significantly increases neurite extension, as well as neuronal branching, in spiral ganglion cell cultures derived from early postnatal rats...
March 21, 2018: Neuroreport
https://www.readbyqxmd.com/read/29567975/loss-of-idh2-accelerates-age-related-hearing-loss-in-male-mice
#11
Karessa White, Mi-Jung Kim, Chul Han, Hyo-Jin Park, Dalian Ding, Kevin Boyd, Logan Walker, Paul Linser, Zaimary Meneses, Cole Slade, Jonathan Hirst, Katherine Santostefano, Naohiro Terada, Takuya Miyakawa, Masaru Tanokura, Richard Salvi, Shinichi Someya
Isocitrate dehydrogenase (IDH) 2 participates in the TCA cycle and catalyzes the conversion of isocitrate to α-ketoglutarate and NADP+ to NADPH. In the mitochondria, IDH2 also plays a key role in protecting mitochondrial components from oxidative stress by supplying NADPH to both glutathione reductase (GSR) and thioredoxin reductase 2 (TXNRD2). Here, we report that loss of Idh2 accelerates age-related hearing loss, the most common form of hearing impairment, in male mice. This was accompanied by increased oxidative DNA damage, increased apoptotic cell death, and profound loss of spiral ganglion neurons and hair cells in the cochlea of 24-month-old Idh2-/- mice...
March 22, 2018: Scientific Reports
https://www.readbyqxmd.com/read/29553844/estimation-of-the-status-of-spiral-ganglion-neurons-and-schwann-cells-in-the-auditory-neural-degeneration-mouse-using-the-auditory-brainstem-response
#12
Zhi-Jian Zhang, Hong-Xia Guan, Kun Yang, Bo-Kui Xiao, Hua Liao, Yang Jiang, Tao Zhou, Qing-Quan Hua
CONCLUSION: The auditory brainstem response (ABR) wave I threshold, latency and amplitude are insensitive to spiral ganglion neurons (SGNs) degeneration, but are sensitive to the degeneration of Schwann cells and can estimate the status of Schwann cells in a neural degeneration mouse model. The thorough pre-operative ABR assessment would be helpful in predicting cochlear implant performance. OBJECTIVES: This study aimed in finding a non-invasive electrophysiological method to evaluate the status of the auditory nerve and the Schwann cells in sensorineural hearing loss (SNHL) and auditory neuropathy (AN) ears, and providing useful information for candidates screening and outcome prediction in cochlear implantation...
March 19, 2018: Acta Oto-laryngologica
https://www.readbyqxmd.com/read/29548764/cabp1-regulates-ca-v-1-l-type-ca-2-channels-and-their-coupling-to-neurite-growth-and-gene-transcription-in-mouse-spiral-ganglion-neurons
#13
Tian Yang, Ji-Eun Choi, Daniel Soh, Kevin Tobin, Mei-Ling Joiner, Marlan Hansen, Amy Lee
CaBP1 is a Ca2+ binding protein that is widely expressed in neurons in the brain, retina, and cochlea. In heterologous expression systems, CaBP1 interacts with and regulates voltage-gated Cav Ca2+ channels but whether this is the case in neurons is unknown. Here, we investigated the cellular functions of CaBP1 in cochlear spiral ganglion neurons (SGNs), which express high levels of CaBP1. Consistent with the role of CaBP1 as a suppressor of Ca2+ -dependent inactivation (CDI) of Cav 1 (L-type) channels, Cav 1 currents underwent greater CDI in SGNs from mice lacking CaBP1 (C-KO) than in wild-type (WT) SGNs...
March 13, 2018: Molecular and Cellular Neurosciences
https://www.readbyqxmd.com/read/29536590/neuronal-cell-adhesion-molecule-nrcam-is-expressed-by-sensory-cells-in-the-cochlea-and-is-necessary-for-proper-cochlear-innervation-and-sensory-domain-patterning-during-development
#14
Randall J Harley, Joseph P Murdy, Zhirong Wang, Michael C Kelly, Tessa-Jonne F Ropp, SeHoon H Park, Patricia F Maness, Paul B Manis, Thomas M Coate
BACKGROUND: In the cochlea, auditory development depends on precise patterns of innervation by afferent and efferent nerve fibers, as well as a stereotyped arrangement of hair and supporting cells. NrCAM is a homophilic cell adhesion molecule that controls diverse aspects of nervous system development, but the function of NrCAM in cochlear development is not well understood. RESULTS: Throughout cochlear innervation, NrCAM is detectable on spiral ganglion neuron (SGN) afferent and olivocochlear efferent fibers, and on the membranes of developing hair and supporting cells...
March 14, 2018: Developmental Dynamics: An Official Publication of the American Association of Anatomists
https://www.readbyqxmd.com/read/29535374/aav-mediated-nt-3-overexpression-protects-cochleae-against-noise-induced-synaptopathy
#15
Hengchao Chen, Yazhi Xing, Li Xia, Zhengnong Chen, Shankai Yin, Jian Wang
The synapse between inner hair cells (IHCs) and type I spiral ganglion neurons (SGNs) has been identified as a sensitive structure to noise-induced damage in the mammalian cochlea. Since this synapse provides the major information pathway from the cochlea to the auditory brain, it is important to maintain its integrity. Neurotrophin-3 (NT-3) has been known to play an important role in the development and the functional maintenance of this synapse. Application of exogenous NT-3, or overexpression of this gene in a transgenic animal model, have shown the value to protect this synapse from noise-induced damage...
March 13, 2018: Gene Therapy
https://www.readbyqxmd.com/read/29512778/mcmv-triggers-ros-nlrp3-associated-inflammasome-activation-in-the-inner-ear-of-mice-and-cultured-spiral-ganglion-neurons-contributing-to-sensorineural-hearing-loss
#16
Wei Zhuang, Caiji Wang, Xi Shi, Shiwei Qiu, Shili Zhang, Bing Xu, Min Chen, Wen Jiang, Hongyan Dong, Yuehua Qiao
Congenital cytomegalovirus (CMV) infection is the most common infectious cause of sensorineural hearing loss in children. While the importance of CMV‑induced SNHL has been described, the mechanisms underlying its pathogenesis and the role of inflammatory responses remain elusive. The present study established an experimental model of hearing loss after systemic infection with murine CMV (MCMV) in newborn mice. Auditory brainstem responses were tested to evaluate hearing at 3 weeks, expression of inflammasome‑-associated factors was assessed by immunofluorescence, western blot analysis, reverse transcription‑quantitative polymerase chain reaction and ELISA...
March 6, 2018: International Journal of Molecular Medicine
https://www.readbyqxmd.com/read/29496363/simulated-auditory-nerve-axon-demyelination-alters-sensitivity-and-response-timing-to-extracellular-stimulation
#17
Jesse M Resnick, Gabrielle E O'Brien, Jay T Rubinstein
Since cochlear implant function involves direct depolarization of spiral ganglion neurons (SGNs) by applied current, SGN physiological health must be an important factor in cochlear implant (CI) outcomes. This expected relationship has, however, been difficult to confirm in implant recipients. Suggestively, animal studies have demonstrated both acute and progressive SGN ultrastructural changes (notably axon demyelination), even in the absence of soma death, and corresponding altered physiology following sensorineural deafening...
February 14, 2018: Hearing Research
https://www.readbyqxmd.com/read/29492404/direct-reprogramming-of-spiral-ganglion-non-neuronal-cells-into-neurons-toward-ameliorating-sensorineural-hearing-loss-by-gene-therapy
#18
Teppei Noda, Steven J Meas, Jumpei Nogami, Yutaka Amemiya, Ryutaro Uchi, Yasuyuki Ohkawa, Koji Nishimura, Alain Dabdoub
Primary auditory neurons (PANs) play a critical role in hearing by transmitting sound information from the inner ear to the brain. Their progressive degeneration is associated with excessive noise, disease and aging. The loss of PANs leads to permanent hearing impairment since they are incapable of regenerating. Spiral ganglion non-neuronal cells (SGNNCs), comprised mainly of glia, are resident within the modiolus and continue to survive after PAN loss. These attributes make SGNNCs an excellent target for replacing damaged PANs through cellular reprogramming...
2018: Frontiers in Cell and Developmental Biology
https://www.readbyqxmd.com/read/29487598/macrophages-in-the-human-cochlea-saviors-or-predators-a-study-using-super-resolution-immunohistochemistry
#19
Wei Liu, Matyas Molnar, Carolyn Garnham, Heval Benav, Helge Rask-Andersen
The human inner ear, which is segregated by a blood/labyrinth barrier, contains resident macrophages [CD163, ionized calcium-binding adaptor molecule 1 (IBA1)-, and CD68-positive cells] within the connective tissue, neurons, and supporting cells. In the lateral wall of the cochlea, these cells frequently lie close to blood vessels as perivascular macrophages. Macrophages are also shown to be recruited from blood-borne monocytes to damaged and dying hair cells induced by noise, ototoxic drugs, aging, and diphtheria toxin-induced hair cell degeneration...
2018: Frontiers in Immunology
https://www.readbyqxmd.com/read/29485861/bisphosphonate-linked-trkb-agonist-cochlea-targeted-delivery-of-a-neurotrophic-agent-as-a-strategy-for-the-treatment-of-hearing-loss
#20
Judith S Kempfle, Kim Nguyen, Christine Hamadani, Nicholas Koen, Albert S Edge, Boris A Kashemirov, David H Jung, Charles E McKenna
Hearing loss affects more than two-thirds of the elderly population, and more than 17% of all adults in the U.S. Sensorineural hearing loss related to noise exposure or aging is associated with loss of inner ear sensory hair cells (HCs), cochlear spiral ganglion neurons (SGNs), and ribbon synapses between HCs and SGNs, stimulating intense interest in therapies to regenerate synaptic function. 7,8-Dihydroxyflavone (DHF) is a selective and potent agonist of tropomyosin receptor kinase B (TrkB) and protects the neuron from apoptosis...
February 27, 2018: Bioconjugate Chemistry
keyword
keyword
96345
1
2
Fetch more papers »
Fetching more papers... Fetching...
Read by QxMD. Sign in or create an account to discover new knowledge that matter to you.
Remove bar
Read by QxMD icon Read
×

Search Tips

Use Boolean operators: AND/OR

diabetic AND foot
diabetes OR diabetic

Exclude a word using the 'minus' sign

Virchow -triad

Use Parentheses

water AND (cup OR glass)

Add an asterisk (*) at end of a word to include word stems

Neuro* will search for Neurology, Neuroscientist, Neurological, and so on

Use quotes to search for an exact phrase

"primary prevention of cancer"
(heart or cardiac or cardio*) AND arrest -"American Heart Association"