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https://www.readbyqxmd.com/read/28925723/expression-patterns-of-members-of-the-isocitrate-dehydrogenase-gene-family-in-murine-inner-ear
#1
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
#2
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
#3
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
#4
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/28899994/heterodimeric-capping-protein-is-required-for-stereocilia-length-and-width-regulation
#5
Matthew R Avenarius, Jocelyn F Krey, Rachel A Dumont, Clive P Morgan, Connor B Benson, Sarath Vijayakumar, Christopher L Cunningham, Deborah I Scheffer, David P Corey, Ulrich Müller, Sherri M Jones, Peter G Barr-Gillespie
Control of the dimensions of actin-rich processes like filopodia, lamellipodia, microvilli, and stereocilia requires the coordinated activity of many proteins. Each of these actin structures relies on heterodimeric capping protein (CAPZ), which blocks actin polymerization at barbed ends. Because dimension control of the inner ear's stereocilia is particularly precise, we studied the CAPZB subunit in hair cells. CAPZB, present at ∼100 copies per stereocilium, concentrated at stereocilia tips as hair cell development progressed, similar to the CAPZB-interacting protein TWF2...
September 12, 2017: Journal of Cell Biology
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/28866362/gene-cell-and-organ-multiplication-drives-inner-ear-evolution
#7
REVIEW
Bernd Fritzsch, Karen L Elliott
We review the development and evolution of the ear neurosensory cells, the aggregation of neurosensory cells into an otic placode, the evolution of novel neurosensory structures dedicated to hearing and the evolution of novel nuclei in the brain and their input dedicated to processing those novel auditory stimuli. The evolution of the apparently novel auditory system lies in duplication and diversification of cell fate transcription regulation that allows variation at the cellular level [transforming a single neurosensory cell into a sensory cell connected to its targets by a sensory neuron as well as diversifying hair cells], organ level [duplication of organ development followed by diversification and novel stimulus acquisition] and brain nuclear level [multiplication of transcription factors to regulate various neuron and neuron aggregate fate to transform the spinal cord into the unique hindbrain organization]...
August 31, 2017: Developmental Biology
https://www.readbyqxmd.com/read/28855028/fgf-signaling-diverse-roles-during-cochlear-development
#8
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
#9
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
#10
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
#11
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/28838968/tbx1-and-jag1-act-in-concert-to-modulate-the-fate-of-neurosensory-cells-of-the-mouse-otic-vesicle
#12
Stephania Macchiarulo, Bernice E Morrow
The domain within the otic vesicle (OV) known as the neurosensory domain (NSD), contains cells that will give rise to the hair and support cells of the otic sensory organs, as well as the neurons that form the cochleovestibular ganglion (CVG). The molecular dynamics that occur at the NSD boundary relative to adjacent OV cells is not well defined. The Tbx1 transcription factor gene expression pattern is complementary to the NSD, and inactivation results in an expansion of the NSD and expression of the Notch ligand, Jag1 mapping, in part to the NSD...
August 24, 2017: Biology Open
https://www.readbyqxmd.com/read/28835534/local-gene-therapy-durably-restores-vestibular-function-in-a-mouse-model-of-usher-syndrome-type-1g
#13
Alice Emptoz, Vincent Michel, Andrea Lelli, Omar Akil, Jacques Boutet de Monvel, Ghizlene Lahlou, Anaïs Meyer, Typhaine Dupont, Sylvie Nouaille, Elody Ey, Filipa Franca de Barros, Mathieu Beraneck, Didier Dulon, Jean-Pierre Hardelin, Lawrence Lustig, Paul Avan, Christine Petit, Saaid Safieddine
Our understanding of the mechanisms underlying inherited forms of inner ear deficits has considerably improved during the past 20 y, but we are still far from curative treatments. We investigated gene replacement as a strategy for restoring inner ear functions in a mouse model of Usher syndrome type 1G, characterized by congenital profound deafness and balance disorders. These mice lack the scaffold protein sans, which is involved both in the morphogenesis of the stereociliary bundle, the sensory antenna of inner ear hair cells, and in the mechanoelectrical transduction process...
September 5, 2017: Proceedings of the National Academy of Sciences of the United States of America
https://www.readbyqxmd.com/read/28834538/claudin7b-is-required-for-the-formation-and-function-of-inner-ear-in-zebrafish
#14
Xiaohui Li, Guili Song, Yasong Zhao, Feng Zhao, Chunyan Liu, Dong Liu, Qing Li, Zongbin Cui
Zebrafish has become an excellent model for studying the development and function of inner ear. We report here a zebrafish line in which claudin 7b (cldn7b) locus is interrupted by a Tol2 transposon at its first intron. The homozygous mutants have enlarged otocysts, smaller or no otoliths, slowly formed semicircular canals, and insensitiveness to sound stimulation. These abnormal phenotypes and hearing loss of inner ear could be mostly rescued by injection of cldn7b-mRNA into one-cell stage homozygous mutant embryos...
August 23, 2017: Journal of Cellular Physiology
https://www.readbyqxmd.com/read/28832620/a-novel-splice-site-mutation-of-myosin-vi-in-mice-leads-to-stereociliary-fusion-caused-by-disruption-of-actin-networks-in-the-apical-region-of-inner-ear-hair-cells
#15
Yuta Seki, Yuki Miyasaka, Sari Suzuki, Kenta Wada, Shumpei P Yasuda, Kunie Matsuoka, Yasuhiro Ohshiba, Kentaro Endo, Rie Ishii, Hiroshi Shitara, Shin-Ichiro Kitajiri, Naomi Nakagata, Hirohide Takebayashi, Yoshiaki Kikkawa
An unconventional myosin encoded by the myosin VI gene (MYO6) contributes to hearing loss in humans. Homozygous mutations of MYO6 result in nonsyndromic profound congenital hearing loss, DFNB37. Kumamoto shaker/waltzer (ksv) mice harbor spontaneous mutations, and homozygous mutants exhibit congenital defects in balance and hearing caused by fusion of the stereocilia. We identified a Myo6c.1381G>A mutation that was found to be a p.E461K mutation leading to alternative splicing errors in Myo6 mRNA in ksv mutants...
2017: PloS One
https://www.readbyqxmd.com/read/28821654/wnt9a-can-influence-cell-fates-and-neural-connectivity-across-the-radial-axis-of-the-developing-cochlea
#16
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/28818455/adipose-derived-stromal-cells-enhance-auditory-neuron-survival-in-an-animal-model-of-sensory-hearing-loss
#17
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
#18
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
https://www.readbyqxmd.com/read/28776202/neurotrophin-gene-therapy-in-deafened-ears-with-cochlear-implants-long-term-effects-on-nerve-survival-and-functional-measures
#19
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/28751305/dlx3b-4b-is-required-for-early-born-but-not-later-forming-sensory-hair-cells-during-zebrafish-inner-ear-development
#20
Simone Schwarzer, Sandra Spieß, Michael Brand, Stefan Hans
Morpholino-mediated knockdown has shown that the homeodomain transcription factors Dlx3b and Dlx4b are essential for proper induction of the otic-epibranchial progenitor domain (OEPD), as well as subsequent formation of sensory hair cells in the developing zebrafish inner ear. However, increasing use of reverse genetic approaches has revealed poor correlation between morpholino-induced and mutant phenotypes. Using CRISPR/Cas9-mediated mutagenesis, we generated a defined deletion eliminating the entire open reading frames of dlx3b and dlx4b (dlx3b/4b) and investigated a potential phenotypic difference between mutants and morpholino-mediated knockdown...
September 15, 2017: Biology Open
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