Search for Articles:
Title / Keyword
Author / Affiliation / Email
Journal
Article Type
 
 
Advanced Search
 
Section
Special Issue
Volume
Issue
Number
Page
 
9.0
6.0
Journals
Cells
Special Issues
Stem Cells and Hearing Loss
share announcement

Stem Cells and Hearing Loss

A special issue of Cells (ISSN 2073-4409). This special issue belongs to the section "Stem Cells".

Deadline for manuscript submissions: closed (15 February 2023) | Viewed by 12548

Special Issue Editors

Prof. Dr. Brigitte Malgrange

E-Mail Website
Guest Editor
GIGA-Stem Cells, GIGA Research Center, University of Liège, LIEGE 4000, Belgium
Interests: inner ear; cochlea; iPS cells; organoids; Stroke; Adult neurogenesis; proliferation; cell death
Dr. Subhajit Giri

E-Mail Website
Guest Editor
GIGA-Stem Cell/Neuroscience, Université de Liège, 4000 Liege, Belgium
Interests: stem cell biology; iPSC based disease modelling; gene editing; CRISPR/Cas

Special Issue Information

Dear Colleagues,

Hearing loss is the most common sensorineural disorder affecting around 6.1% of the world population. It is of utmost importance to find an effective therapeutic intervention with the available cutting-edge research-based tools that can cure or halt disease progression.

This Special Issue is designed to stimulate an understanding of sensorineural hearing loss and provide a platform to exchange new concepts and ideas, focusing on induced pluripotent stem cells (iPSCs) and 3D-organoid-based disease modeling, genetic and epigenetic perturbation through CRISPR, and emerging innovative techniques and protocols. I am delighted to invite you to publish your research articles and review papers in this issue focused on the following domains.

  1. iPSC-based investigation of inner ear development;
    2. Cellular and molecular biology of inner ear development;
    3. 3D-organoid-based disease modeling of hereditary deafness;
    4. iPSC differentiation protocols toward sensory neurons and other inner ear components;
    5. Emerging therapeutic application of CRISPR and iPSC for hereditary deafness;
    6. iPSC-based disease modeling to study the effect(s) of environmental/toxic compound/idiopathic factors;
    7. iPSC-based transdifferentiation toward cochlear hair cells.

We look forward to publishing your exciting and thought-provoking research communications.

Thank you

Prof. Dr. Brigitte Malgrange
Dr. Subhajit Giri

Guest Editors

Manuscript Submission Information

Manuscripts should be submitted online at www.mdpi.com by registering and logging in to this website. Once you are registered, click here to go to the submission form. Manuscripts can be submitted until the deadline. All submissions that pass pre-check are peer-reviewed. Accepted papers will be published continuously in the journal (as soon as accepted) and will be listed together on the special issue website. Research articles, review articles as well as short communications are invited. For planned papers, a title and short abstract (about 100 words) can be sent to the Editorial Office for announcement on this website.

Submitted manuscripts should not have been published previously, nor be under consideration for publication elsewhere (except conference proceedings papers). All manuscripts are thoroughly refereed through a single-blind peer-review process. A guide for authors and other relevant information for submission of manuscripts is available on the Instructions for Authors page. Cells is an international peer-reviewed open access semimonthly journal published by MDPI.

Please visit the Instructions for Authors page before submitting a manuscript. The Article Processing Charge (APC) for publication in this open access journal is 2700 CHF (Swiss Francs). Submitted papers should be well formatted and use good English. Authors may use MDPI's English editing service prior to publication or during author revisions.

Keywords

  • Crispr/cas9
  • reprogramming
  • stem cells
  • nervous system
  • epigenetic

Published Papers (5 papers)

Download All Papers
Order results
Result details
Show export options expand_more Show export options expand_less
Select all
Export citation of selected articles as:

Research

Jump to: Review

18 pages, 5445 KiB  
Article
The Protective Effects of Human Embryonic Stem Cell-Derived Mesenchymal Stem Cells in Noise-Induced Hearing Loss of Rats
by So Young Kim, Jeoung Eun Lee, Sung Hun Kang, So Min Lee, Jiwon Jeon and Dong Ryul Lee
Cells 2022, 11(21), 3524; https://doi.org/10.3390/cells11213524 - 07 Nov 2022
Cited by 1 | Viewed by 1770
Abstract
A few prior animal studies have suggested the transplantation or protective effects of mesenchymal stem cells (MSCs) in noise-induced hearing loss. This study intended to evaluate the fates of administered MSCs in the inner ears and the otoprotective effects of MSCs in the [...] Read more.
A few prior animal studies have suggested the transplantation or protective effects of mesenchymal stem cells (MSCs) in noise-induced hearing loss. This study intended to evaluate the fates of administered MSCs in the inner ears and the otoprotective effects of MSCs in the noise-induced hearing loss of rats. Human embryonic stem cell-derived MSCs (ES-MSCs) were systematically administered via the tail vein in adult rats. Eight-week-old Sprague-Dawley rats were randomly allocated to the control (n = 8), ES-MSC (n = 4), noise (n = 8), and ES-MSC+noise (n = 10) groups. In ES-MSC and ES-MSC+noise rats, 5 × 105 ES-MSCs were injected via the tail vein. In noise and ES-MSC+noise rats, broadband noise with 115 dB SPL was exposed for 3 h daily for 5 days. The hearing levels were measured using auditory brainstem response (ABR) at 4, 8, 16, and 32 kHz. Cochlear histology was examined using H&E staining and cochlear whole mount immunofluorescence. The presence of human DNA was examined using Sry PCR, and the presence of human cytoplasmic protein was examined using STEM121 immunofluorescence staining. The protein expression levels of heat shock protein 70 (HSP70), apoptosis-inducing factor (AIF), poly (ADP-ribose) (PAR), PAR polymerase (PARP), caspase 3, and cleaved caspase 3 were estimated. The ES-MSC rats did not show changes in ABR thresholds following the administration of ES-MSCs. The ES-MSC+ noise rats demonstrated lower ABR thresholds at 4, 8, and 16 kHz than the noise rats. Cochlear spiral ganglial cells and outer hair cells were more preserved in the ES-MSC+ noise rats than in the noise rats. The Sry PCR bands were highly detected in lung tissue and less in cochlear tissue of ES-MSC+noise rats. Only a few STEM121-positivities were observed in the spiral ganglial cell area of ES-MSC and ES-MSC+noise rats. The protein levels of AIF, PAR, PARP, caspase 3, and cleaved caspase 3 were lower in the ES-MSC+noise rats than in the noise rats. The systemic injection of ES-MSCs preserved hearing levels and attenuated parthanatos and apoptosis in rats with noise-induced hearing loss. In addition, a tiny number of transplanted ES-MSCs were observed in the spiral ganglial areas. Full article
(This article belongs to the Special Issue Stem Cells and Hearing Loss)
►▼ Show Figures

Figure 1

20 pages, 5199 KiB  
Article
WNT Activation and TGFβ-Smad Inhibition Potentiate Stemness of Mammalian Auditory Neuroprogenitors for High-Throughput Generation of Functional Auditory Neurons In Vitro
by Francis Rousset, Giulia Schilardi, Stéphanie Sgroi, German Nacher-Soler, Rebecca Sipione, Sonja Kleinlogel and Pascal Senn
Cells 2022, 11(15), 2431; https://doi.org/10.3390/cells11152431 - 05 Aug 2022
Viewed by 1931
Abstract
Hearing loss affects over 460 million people worldwide and is a major socioeconomic burden. Both genetic and environmental factors (i.e., noise overexposure, ototoxic drug treatment and ageing), promote the irreversible degeneration of cochlear hair cells and associated auditory neurons, leading to sensorineural hearing [...] Read more.
Hearing loss affects over 460 million people worldwide and is a major socioeconomic burden. Both genetic and environmental factors (i.e., noise overexposure, ototoxic drug treatment and ageing), promote the irreversible degeneration of cochlear hair cells and associated auditory neurons, leading to sensorineural hearing loss. In contrast to birds, fish and amphibians, the mammalian inner ear is virtually unable to regenerate due to the limited stemness of auditory progenitors, and no causal treatment is able to prevent or reverse hearing loss. As of today, a main limitation for the development of otoprotective or otoregenerative therapies is the lack of efficient preclinical models compatible with high-throughput screening of drug candidates. Currently, the research field mainly relies on primary organotypic inner ear cultures, resulting in high variability, low throughput, high associated costs and ethical concerns. We previously identified and characterized the phoenix auditory neuroprogenitors (ANPGs) as highly proliferative progenitor cells isolated from the A/J mouse cochlea. In the present study, we aim at identifying the signaling pathways responsible for the intrinsic high stemness of phoenix ANPGs. A transcriptomic comparison of traditionally low-stemness ANPGs, isolated from C57Bl/6 and A/J mice at early passages, and high-stemness phoenix ANPGs was performed, allowing the identification of several differentially expressed pathways. Based on differentially regulated pathways, we developed a reprogramming protocol to induce high stemness in presenescent ANPGs (i.e., from C57Bl6 mouse). The pharmacological combination of the WNT agonist (CHIR99021) and TGFβ/Smad inhibitors (LDN193189 and SB431542) resulted in a dramatic increase in presenescent neurosphere growth, and the possibility to expand ANPGs is virtually limitless. As with the phoenix ANPGs, stemness-induced ANPGs could be frozen and thawed, enabling distribution to other laboratories. Importantly, even after 20 passages, stemness-induced ANPGs retained their ability to differentiate into electrophysiologically mature type I auditory neurons. Both stemness-induced and phoenix ANPGs resolve a main bottleneck in the field, allowing efficient, high-throughput, low-cost and 3R-compatible in vitro screening of otoprotective and otoregenerative drug candidates. This study may also add new perspectives to the field of inner ear regeneration. Full article
(This article belongs to the Special Issue Stem Cells and Hearing Loss)
►▼ Show Figures

Graphical abstract

Review

Jump to: Research

16 pages, 2780 KiB  
Review
Application of Human Stem Cells to Model Genetic Sensorineural Hearing Loss and Meniere Disease
by Mar Lamolda, Lidia Frejo, Alvaro Gallego-Martinez and Jose A. Lopez-Escamez
Cells 2023, 12(7), 988; https://doi.org/10.3390/cells12070988 - 23 Mar 2023
Cited by 1 | Viewed by 2281
Abstract
Genetic sensorineural hearing loss and Meniere disease have been associated with rare variations in the coding and non-coding region of the human genome. Most of these variants were classified as likely pathogenic or variants of unknown significance and require functional validation in cellular [...] Read more.
Genetic sensorineural hearing loss and Meniere disease have been associated with rare variations in the coding and non-coding region of the human genome. Most of these variants were classified as likely pathogenic or variants of unknown significance and require functional validation in cellular or animal models. Given the difficulties to obtain human samples and the raising concerns about animal experimentation, human-induced pluripotent stem cells emerged as cellular models to investigate the interaction of genetic and environmental factors in the pathogenesis of inner ear disorders. The generation of human sensory epithelia and neuron-like cells carrying the variants of interest may facilitate a better understanding of their role during differentiation. These cellular models will allow us to explore new strategies for restoring hearing and vestibular sensory epithelia as well as neurons. This review summarized the use of human-induced pluripotent stem cells in sensorineural hearing loss and Meniere disease and proposed some strategies for its application in clinical practice. Full article
(This article belongs to the Special Issue Stem Cells and Hearing Loss)
►▼ Show Figures

Graphical abstract

13 pages, 947 KiB  
Review
ATP-Dependent Chromatin Remodellers in Inner Ear Development
by Ilyas Chohra, Keshi Chung, Subhajit Giri and Brigitte Malgrange
Cells 2023, 12(4), 532; https://doi.org/10.3390/cells12040532 - 07 Feb 2023
Cited by 3 | Viewed by 2492
Abstract
During transcription, DNA replication and repair, chromatin structure is constantly modified to reveal specific genetic regions and allow access to DNA-interacting enzymes. ATP-dependent chromatin remodelling complexes use the energy of ATP hydrolysis to modify chromatin architecture by repositioning and rearranging nucleosomes. These complexes [...] Read more.
During transcription, DNA replication and repair, chromatin structure is constantly modified to reveal specific genetic regions and allow access to DNA-interacting enzymes. ATP-dependent chromatin remodelling complexes use the energy of ATP hydrolysis to modify chromatin architecture by repositioning and rearranging nucleosomes. These complexes are defined by a conserved SNF2-like, catalytic ATPase subunit and are divided into four families: CHD, SWI/SNF, ISWI and INO80. ATP-dependent chromatin remodellers are crucial in regulating development and stem cell biology in numerous organs, including the inner ear. In addition, mutations in genes coding for proteins that are part of chromatin remodellers have been implicated in numerous cases of neurosensory deafness. In this review, we describe the composition, structure and functional activity of these complexes and discuss how they contribute to hearing and neurosensory deafness. Full article
(This article belongs to the Special Issue Stem Cells and Hearing Loss)
►▼ Show Figures

Figure 1

30 pages, 491 KiB  
Review
Induced Pluripotent Stem Cells, a Stepping Stone to In Vitro Human Models of Hearing Loss
by María Beatriz Durán-Alonso and Hrvoje Petković
Cells 2022, 11(20), 3331; https://doi.org/10.3390/cells11203331 - 21 Oct 2022
Cited by 4 | Viewed by 2196
Abstract
Hearing loss is the most prevalent sensorineural impairment in humans. Yet despite very active research, no effective therapy other than the cochlear implant has reached the clinic. Main reasons for this failure are the multifactorial nature of the disorder, its heterogeneity, and a [...] Read more.
Hearing loss is the most prevalent sensorineural impairment in humans. Yet despite very active research, no effective therapy other than the cochlear implant has reached the clinic. Main reasons for this failure are the multifactorial nature of the disorder, its heterogeneity, and a late onset that hinders the identification of etiological factors. Another problem is the lack of human samples such that practically all the work has been conducted on animals. Although highly valuable data have been obtained from such models, there is the risk that inter-species differences exist that may compromise the relevance of the gathered data. Human-based models are therefore direly needed. The irruption of human induced pluripotent stem cell technologies in the field of hearing research offers the possibility to generate an array of otic cell models of human origin; these may enable the identification of guiding signalling cues during inner ear development and of the mechanisms that lead from genetic alterations to pathology. These models will also be extremely valuable when conducting ototoxicity analyses and when exploring new avenues towards regeneration in the inner ear. This review summarises some of the work that has already been conducted with these cells and contemplates future possibilities. Full article
(This article belongs to the Special Issue Stem Cells and Hearing Loss)
Show export options expand_more Show export options expand_less
Select all
Export citation of selected articles as:
 
Displaying articles 1-5
Back to TopTop