scholarly journals Basic helix–loop–helix gene Hes6 delineates the sensory hair cell lineage in the inner ear

2006 ◽  
Vol 235 (6) ◽  
pp. 1689-1700 ◽  
Author(s):  
Dong Qian ◽  
Kristen Radde-Gallwitz ◽  
Michael Kelly ◽  
Björn Tyrberg ◽  
Jaesang Kim ◽  
...  
Keyword(s):  
Hair Cell ◽  
Inner Ear ◽  
Cell Lineage ◽  
Sensory Hair ◽  
Basic Helix Loop Helix ◽  
Helix Loop Helix ◽  
Sensory Hair Cell

scholarly journals Isolation of sensory hair cell specific exosomes in human perilymph

2021 ◽  
Author(s):  
Pei Zhuang ◽  
Suiching Phung ◽  
Athanasia Warnecke ◽  
Alexandra Arambula ◽  
Madeleine St. Peter ◽  
...  
Keyword(s):  
Hearing Loss ◽  
Hair Cell ◽  
Sensorineural Hearing Loss ◽  
Inner Ear ◽  
Molecular Characterization ◽  
Sensorineural Hearing ◽  
Alternative Methods ◽  
Sensory Hair ◽  
Sensory Hair Cell ◽  
Human Inner Ear

AbstractEvaluation of hearing loss patients using clinical audiometry has been unable to give a definitive cellular or molecular diagnosis, hampering the development of treatments of sensorineural hearing loss. However, biopsy of inner ear tissue without losing residual hearing function for pathologic diagnosis is extremely challenging. In a clinical setting, perilymph can be accessed, so alternative methods for molecular characterization of the inner ear may be developed. Recent approaches to improving inner ear diagnostics have been focusing on the evaluation of the proteomic or miRNA profiles of perilymph. Inspired by recent characterization and classification of many neurodegenerative diseases using exosomes which not only are produced in locally in diseased tissue but are transported beyond the blood brain barrier, we demonstrate the isolation of human inner ear specific exosomes using a novel ultrasensitive immunomagnetic nano pom-poms capture-release approach. Using perilymph samples harvested from surgical procedures, we were able to isolate exosomes from sensorineural hearing loss patients in only 2-5 μL of perilymph. By isolating sensory hair cell derived exosomes through their expression level of myosin VII, we for the first time sample material from hair cells in the living human inner ear. This work sets up the first demonstration of immunomagnetic capture-release nano pom-pom isolated exosomes for liquid biopsy diagnosis of sensorineural hearing loss. With the ability to isolate exosomes derived from different cell types for molecular characterization, this method also can be developed for analyzing exosomal biomarkers from more accessible patient tissue fluids such as plasma.

scholarly journals Exosomes mediate sensory hair cell protection in the inner ear

2020 ◽  
Vol 130 (5) ◽  
pp. 2657-2672 ◽  
Author(s):  
Andrew M. Breglio ◽  
Lindsey A. May ◽  
Melanie Barzik ◽  
Nora C. Welsh ◽  
Shimon P. Francis ◽  
...  
Keyword(s):  
Hair Cell ◽  
Inner Ear ◽  
Cell Protection ◽  
Sensory Hair ◽  
Sensory Hair Cell

scholarly journals A missense mutation in DCDC2 causes human recessive deafness DFNB66, likely by interfering with sensory hair cell and supporting cell cilia length regulation

2015 ◽  
Vol 24 (9) ◽  
pp. 2482-2491 ◽  
Author(s):  
M'hamed Grati ◽  
Imen Chakchouk ◽  
Qi Ma ◽  
Mariem Bensaid ◽  
Alexandra Desmidt ◽  
...  
Keyword(s):  
Hair Cell ◽  
Missense Mutation ◽  
Supporting Cell ◽  
Sensory Hair ◽  
Sensory Hair Cell ◽  
Length Regulation

scholarly journals Dimethyl Sulfoxide (DMSO) Exacerbates Cisplatin-induced Sensory Hair Cell Death in Zebrafish (Danio rerio)

PLoS ONE ◽  
2013 ◽  
Vol 8 (2) ◽  
pp. e55359 ◽  
Author(s):  
Phillip M. Uribe ◽  
Melissa A. Mueller ◽  
Julia S. Gleichman ◽  
Matthew D. Kramer ◽  
Qi Wang ◽  
...  
Keyword(s):  
Cell Death ◽  
Hair Cell ◽  
Dimethyl Sulfoxide ◽  
Danio Rerio ◽  
Sensory Hair ◽  
Hair Cell Death ◽  
Sensory Hair Cell

NeuroD-null mice are deaf due to a severe loss of the inner ear sensory neurons during development

Development ◽  
2001 ◽  
Vol 128 (3) ◽  
pp. 417-426 ◽  
Author(s):  
W.Y. Kim ◽  
B. Fritzsch ◽  
A. Serls ◽  
L.A. Bakel ◽  
E.J. Huang ◽  
...  
Keyword(s):  
Inner Ear ◽  
Sensory Neurons ◽  
Auditory Evoked Potentials ◽  
Neurotrophin Receptors ◽  
Basic Helix Loop Helix ◽  
Helix Loop Helix ◽  
Developmental Neuroscience ◽  
Profound Deafness ◽  
Key Factor ◽  
Genetic Mechanisms

A key factor in the genetically programmed development of the nervous system is the death of massive numbers of neurons. Therefore, genetic mechanisms governing cell survival are of fundamental importance to developmental neuroscience. We report that inner ear sensory neurons are dependent on a basic helix-loop-helix transcription factor called NeuroD for survival during differentiation. Mice lacking NeuroD protein exhibit no auditory evoked potentials, reflecting a profound deafness. DiI fiber staining, immunostaining and cell death assays reveal that the deafness is due to the failure of inner ear sensory neuron survival during development. The affected inner ear sensory neurons fail to express neurotrophin receptors, TrkB and TrkC, suggesting that the ability of NeuroD to support neuronal survival may be directly mediated through regulation of responsiveness to the neurotrophins.

scholarly journals Oral Antioxidant Vitamins and Magnesium Limit Noise-Induced Hearing Loss by Promoting Sensory Hair Cell Survival: Role of Antioxidant Enzymes and Apoptosis Genes

Antioxidants ◽  
2020 ◽  
Vol 9 (12) ◽  
pp. 1177
Author(s):  
Juan C. Alvarado ◽  
Verónica Fuentes-Santamaría ◽  
Pedro Melgar-Rojas ◽  
María C. Gabaldón-Ull ◽  
José J. Cabanes-Sanchis ◽  
...  
Keyword(s):  
Cell Death ◽  
Hearing Loss ◽  
Antioxidant Enzymes ◽  
Oral Administration ◽  
Hair Cell ◽  
Cell Survival ◽  
Sensory Cell ◽  
Auditory Function ◽  
Sensory Hair ◽  
Sensory Hair Cell

Noise induces oxidative stress in the cochlea followed by sensory cell death and hearing loss. The proof of principle that injections of antioxidant vitamins and Mg2+ prevent noise-induced hearing loss (NIHL) has been established. However, effectiveness of oral administration remains controversial and otoprotection mechanisms are unclear. Using auditory evoked potentials, quantitative PCR, and immunocytochemistry, we explored effects of oral administration of vitamins A, C, E, and Mg2+ (ACEMg) on auditory function and sensory cell survival following NIHL in rats. Oral ACEMg reduced auditory thresholds shifts after NIHL. Improved auditory function correlated with increased survival of sensory outer hair cells. In parallel, oral ACEMg modulated the expression timeline of antioxidant enzymes in the cochlea after NIHL. There was increased expression of glutathione peroxidase-1 and catalase at 1 and 10 days, respectively. Also, pro-apoptotic caspase-3 and Bax levels were diminished in ACEMg-treated rats, at 10 and 30 days, respectively, following noise overstimulation, whereas, at day 10 after noise exposure, the levels of anti-apoptotic Bcl-2, were significantly increased. Therefore, oral ACEMg improves auditory function by limiting sensory hair cell death in the auditory receptor following NIHL. Regulation of the expression of antioxidant enzymes and apoptosis-related proteins in cochlear structures is involved in such an otoprotective mechanism.

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