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spiral ganglion neuron

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https://www.readbyqxmd.com/read/29033307/neurog1-regulates-cdk2-to-promote-proliferation-in-otic-progenitors
#1
Zhichao Song, Azadeh Jadali, Bernd Fritzsch, Kelvin Y Kwan
Loss of spiral ganglion neurons (SGNs) significantly contributes to hearing loss. Otic progenitor cell transplantation is a potential strategy to replace lost SGNs. Understanding how key transcription factors promote SGN differentiation in otic progenitors accelerates efforts for replacement therapies. A pro-neural transcription factor, Neurogenin1 (Neurog1), is essential for SGN development. Using an immortalized multipotent otic progenitor (iMOP) cell line that can self-renew and differentiate into otic neurons, NEUROG1 was enriched at the promoter of cyclin-dependent kinase 2 (Cdk2) and neurogenic differentiation 1 (NeuroD1) genes...
October 11, 2017: Stem Cell Reports
https://www.readbyqxmd.com/read/28941704/mir-194-is-involved-in-morphogenesis-of-spiral-ganglion-neurons-in-inner-ear-by-rearranging-actin-cytoskeleton-via-targeting-rhob
#2
Jintao Du, Xuemei Zhang, Hui Cao, Di Jiang, Xianren Wang, Wei Zhou, Kaitian Chen, Jiao Zhou, Hongyan Jiang, Luo Ba
Many microRNAs participate in the development, differentiation and function preservation of the embryonic and adult inner ear, but many details still need to be elucidated regarding the numerous microRNAs in the inner ear. Based on previous investigations on the microRNA profile in the inner ear, we confirmed that several microRNAs are expressed in the inner ear, and we detected the spatial expression of these microRNAs in the neonatal mouse inner ear. Then we focused on miR-194 for its specific expression with a dynamic spatiotemporal pattern during inner ear development...
September 20, 2017: International Journal of Developmental Neuroscience
https://www.readbyqxmd.com/read/28925723/expression-patterns-of-members-of-the-isocitrate-dehydrogenase-gene-family-in-murine-inner-ear
#3
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/28893864/the-beach-protein-lrba-is-required-for-hair-bundle-maintenance-in-cochlear-hair-cells-and-for-hearing
#4
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/28889125/brimonidine-protects-auditory-hair-cells-from-in-vitro-induced-toxicity-of-gentamicin
#5
Maurizio Cortada, Soledad Levano, Daniel Bodmer
Brimonidine, an alpha-2 adrenergic receptor (α2-AR) agonist, has neuroprotective effects in the visual system and in spiral ganglion neurons. Auditory hair cells (HCs) express all 3 α2-AR subtypes, but their roles in HCs remain unknown. This study investigated the effects of brimonidine on auditory HCs that were also exposed to gentamicin, which is toxic to HCs. Organ of Corti explants were exposed to gentamicin in the presence or absence of brimonidine, and the α2-AR protein expression levels and Erk1/2 and Akt phosphorylation levels were determined...
September 9, 2017: Audiology & Neuro-otology
https://www.readbyqxmd.com/read/28878625/non-autonomous-cellular-responses-to-ototoxic-drug-induced-stress-and-death
#6
REVIEW
Shimon P Francis, Lisa L Cunningham
The first major recognition of drug-induced hearing loss can be traced back more than seven decades to the development of streptomycin as an antimicrobial agent. Since then at least 130 therapeutic drugs have been recognized as having ototoxic side-effects. Two important classes of ototoxic drugs are the aminoglycoside antibiotics and the platinum-based antineoplastic agents. These drugs save the lives of millions of people worldwide, but they also cause irreparable hearing loss. In the inner ear, sensory hair cells (HCs) and spiral ganglion neurons (SGNs) are important cellular targets of these drugs, and most mechanistic studies have focused on the cell-autonomous responses of these cell types in response to ototoxic stress...
2017: Frontiers in Cellular Neuroscience
https://www.readbyqxmd.com/read/28859106/local-inner-ear-application-of-dexamethasone-in-cochlear-implant-models-is-safe-for-auditory-neurons-and-increases-the-neuroprotective-effect-of-chronic-electrical-stimulation
#7
Verena Scheper, Roland Hessler, Mareike Hütten, Maciej Wilk, Claude Jolly, Thomas Lenarz, Gerrit Paasche
Dexamethasone (DEX) can reduce fibrous tissue growth as well as loss of residual hearing which may occur after cochlear implantation. Little is known about the effect of local inner ear DEX treatment on the spiral ganglion neurons (SGN), which are the target of the electrical stimulation with a cochlear implant (CI). Three different clinically relevant strategies of DEX-delivery into the inner ear were used. DEX was either eluted from the electrode carriers' silicone, released from a reservoir by passive diffusion, or actively applied using a pump based system...
2017: PloS One
https://www.readbyqxmd.com/read/28852025/intravenous-raav2-9-injection-for-murine-cochlear-gene-delivery
#8
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/28841276/tuning-surface-and-topographical-features-to-investigate-competitive-guidance-of-spiral-ganglion-neurons
#9
Braden L Leigh, Kristy Truong, Reid Bartholomew, Mark Ramirez, Marlan R Hansen, C Allan Guymon
Cochlear Implants (CIs) suffer from limited tonal resolution due, in large part, to spatial separation between stimulating electrode arrays and primary neural receptors. In this work, a combination of physical and chemical micropatterns, formed on acrylate polymers, are used to direct the growth of primary spiral ganglion neurons (SGNs), the inner ear neurons. Utilizing the inherent temporal and spatial control of photopolymerization, physical microgrooves are fabricated using a photomask in a single step process...
September 20, 2017: ACS Applied Materials & Interfaces
https://www.readbyqxmd.com/read/28837644/prickle1-regulates-neurite-outgrowth-of-apical-spiral-ganglion-neurons-but-not-hair-cell-polarity-in-the-murine-cochlea
#10
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/28819108/spag6-mutant-mice-have-defects-in-development-and-function-of-spiral-ganglion-neurons-apoptosis-and-higher-sensitivity-to-paclitaxel
#11
Xiaofei Li, Lei Xu, Gaoying Sun, Xianmin Wu, Xiaohui Bai, Jianfeng Li, Jerome F Strauss, Zhibing Zhang, Haibo Wang
Mammalian Sperm Associated Antigen 6 (SPAG6) is the orthologue of Chlamydomonas PF16, a protein localized in the axoneme central apparatus. Recent studies showed that Spag6 has a role in brain neuronal proliferation and differentiation. The mammalian spiral ganglion neurons (SGNs) are specialzed bipolar neurons in the inner ear. However, the role of SPAG6 in SGN has not been elucidated. Therefore, We hypothesized that a Spag6 knockout would affect the development and function of SGNs. We utilized Spag6-deficient mice and SGN explants to define the role of SPAG6...
August 17, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28780929/a-spiral-attractor-network-drives-rhythmic-locomotion
#12
Angela M Bruno, William N Frost, Mark D Humphries
The joint activity of neural populations is high dimensional and complex. One strategy for reaching a tractable understanding of circuit function is to seek the simplest dynamical system that can account for the population activity. By imaging Aplysia's pedal ganglion during fictive locomotion, here we show that its population-wide activity arises from a low-dimensional spiral attractor. Evoking locomotion moved the population into a low-dimensional, periodic, decaying orbit - a spiral - in which it behaved as a true attractor, converging to the same orbit when evoked, and returning to that orbit after transient perturbation...
August 7, 2017: ELife
https://www.readbyqxmd.com/read/28776202/neurotrophin-gene-therapy-in-deafened-ears-with-cochlear-implants-long-term-effects-on-nerve-survival-and-functional-measures
#13
Bryan E Pfingst, Deborah J Colesa, Donald L Swiderski, Aaron P Hughes, Stefan B Strahl, Moaz Sinan, Yehoash Raphael
Because cochlear implants function by stimulating the auditory nerve, it is assumed that the condition of the nerve plays an important role in the efficacy of the prosthesis. Thus, considerable research has been devoted to methods of preserving the nerve following deafness. Neurotrophins have been identified as a potential contributor to neural health, but most of the research to date has been done in young animals and for short periods (less than 3 to 6 months) after the onset of treatment. The first objective of the current experiment was to examine the effects of a neurotrophin gene therapy delivery method on spiral ganglion neuron (SGN) preservation and function in the long term (5 to 14 months) in mature guinea pigs with cochlear implants...
August 3, 2017: Journal of the Association for Research in Otolaryngology: JARO
https://www.readbyqxmd.com/read/28766844/timing-of-cochlear-implantation-in-auditory-neuropathy-patients-with-otof-mutations-our-experience-with-10-patients
#14
C C Wu, C J Hsu, F L Huang, Y H Lin, Y H Lin, T C Liu, C M Wu
No abstract text is available yet for this article.
August 2, 2017: Clinical Otolaryngology
https://www.readbyqxmd.com/read/28758056/single-cell-transcriptome-analysis-of-developing-and-regenerating-spiral-ganglion-neurons
#15
Kelvin Y Kwan
The spiral ganglion neurons (SGNs) of the cochlea are essential for our ability to hear. SGN loss after exposure to ototoxic drugs or loud noise results in hearing loss. Pluripotent stem cell-derived and endogenous progenitor cell types have the potential to become SGNs and are cellular foundations for replacement therapies. Repurposing transcriptional regulatory networks to promote SGN differentiation from progenitor cells is a strategy for regeneration. Advances in the Fludigm C1 workflow or Drop-seq allow sequencing of single cell transcriptomes to reveal variability between cells...
October 2016: Current Pharmacology Reports
https://www.readbyqxmd.com/read/28753740/photopolymerized-micropatterns-with-high-feature-frequencies-overcome-chemorepulsive-borders-to-direct-neurite-growth
#16
Bradley W Tuft, Linjing Xu, Braden Leigh, Daniel Lee, C Allan Guymon, Marlan R Hansen
Developing and regenerating neurites respond to a variety of biophysical and biochemical cues in their microenvironment to reach target cells and establish appropriate synapses. Defining the hierarchal relationship of both types of cues to direct neurite growth carries broad significance for neural development, regeneration, and, in particular, engineering of neural prostheses that improve tissue integration with native neural networks. In this work, chemorepulsive biochemical borders are established on substrates with a range of surface microfeatures to determine the potential of physical cues to overcome conflicting biochemical cues...
July 28, 2017: Journal of Tissue Engineering and Regenerative Medicine
https://www.readbyqxmd.com/read/28743950/auditory-neuropathy-after-damage-to-cochlear-spiral-ganglion-neurons-in-mice-resulting-from-conditional-expression-of-diphtheria-toxin-receptors
#17
Haolai Pan, Qiang Song, Yanyan Huang, Jiping Wang, Renjie Chai, Shankai Yin, Jian Wang
Auditory neuropathy (AN) is a hearing disorder characterized by normal cochlear amplification to sound but poor temporal processing and auditory perception in noisy backgrounds. These deficits likely result from impairments in auditory neural synchrony; such dyssynchrony of the neural responses has been linked to demyelination of auditory nerve fibers. However, no appropriate animal models are currently available that mimic this pathology. In this study, Cre-inducible diphtheria toxin receptor (iDTR (+/+) ) mice were cross-mated with mice containing Cre (Bhlhb5-Cre (+/-) ) specific to spiral ganglion neurons (SGNs)...
July 25, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28717876/structural-and-ultrastructural-changes-to-type-i-spiral-ganglion-neurons-and-schwann-cells-in-the-deafened-guinea-pig-cochlea
#18
Andrew K Wise, Remy Pujol, Thomas G Landry, James B Fallon, Robert K Shepherd
Sensorineural hearing loss is commonly caused by damage to cochlear sensory hair cells. Coinciding with hair cell degeneration, the peripheral fibres of type I spiral ganglion neurons (SGNs) that normally form synaptic connections with the inner hair cell gradually degenerate. We examined the time course of these degenerative changes in type I SGNs and their satellite Schwann cells at the ultrastructural level in guinea pigs at 2, 6, and 12 weeks following aminoglycoside-induced hearing loss. Degeneration of the peripheral fibres occurred prior to the degeneration of the type I SGN soma and was characterised by shrinkage of the fibre followed by retraction of the axoplasm, often leaving a normal myelin lumen devoid of axoplasmic content...
July 17, 2017: Journal of the Association for Research in Otolaryngology: JARO
https://www.readbyqxmd.com/read/28672008/nanofibrous-scaffolds-for-the-guidance-of-stem-cell-derived-neurons-for-auditory-nerve-regeneration
#19
Sandra Hackelberg, Samuel J Tuck, Long He, Arjun Rastogi, Christina White, Liqian Liu, Diane M Prieskorn, Ryan J Miller, Che Chan, Benjamin R Loomis, Joseph M Corey, Josef M Miller, R Keith Duncan
Impairment of spiral ganglion neurons (SGNs) of the auditory nerve is a major cause for hearing loss occurring independently or in addition to sensory hair cell damage. Unfortunately, mammalian SGNs lack the potential for autonomous regeneration. Stem cell based therapy is a promising approach for auditory nerve regeneration, but proper integration of exogenous cells into the auditory circuit remains a fundamental challenge. Here, we present novel nanofibrous scaffolds designed to guide the integration of human stem cell-derived neurons in the internal auditory meatus (IAM), the foramen allowing passage of the spiral ganglion to the auditory brainstem...
2017: PloS One
https://www.readbyqxmd.com/read/28671816/photopolymerizable-zwitterionic-polymer-patterns-control-cell-adhesion-and-guide-neural-growth
#20
Braden L Leigh, Elise Cheng, Linjing Xu, Corinne Andresen, Marlan R Hansen, C Allan Guymon
Developing materials that reduce or eliminate fibrosis encapsulation of neural prosthetic implants could significantly enhance implant fidelity by improving the tissue/electrode array interface. Here, we report on the photografting and patterning of two zwitterionic materials, sulfobetaine methacrylate (SBMA) and carboxybetaine methacrylate (CBMA), for controlling the adhesion and directionality of cells relevant to neural prosthetics. CBMA and SBMA polymers were photopolymerized and grafted on glass surfaces then characterized by X-ray photoelectron spectroscopy, water contact angle, and protein adsorption...
July 24, 2017: Biomacromolecules
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