Article
Multidisciplinary Sciences
James B. Dewey, Alessandro Altoe, Christopher A. Shera, Brian E. Applegate, John S. Oghalai
Summary: The study found that the electromotility dependent on prestin serves as the primary high-frequency amplifying mechanism in the mammalian cochlea, despite being affected by low-pass filtering in vivo.
PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA
(2021)
Article
Cell Biology
Andre Landin Malt, Shaylyn Clancy, Diane Hwang, Alice Liu, Connor Smith, Margaret Smith, Maya Hatley, Christopher Clemens, Xiaowei Lu
Summary: A non-canonical Wnt signaling pathway has been found to promote cochlear outgrowth and coordinate planar polarization of hair cells. Gsk3 beta and Rac1 play crucial roles in this pathway, as shown in genetic rescue experiments.
FRONTIERS IN CELL AND DEVELOPMENTAL BIOLOGY
(2021)
Article
Audiology & Speech-Language Pathology
Kazuaki Homma, Satoe Takahashi, Mary Ann Cheatham
Summary: Through studying several prestin mouse models, it was found that near-normal cochlear function can be maintained even when prestin activity is significantly reduced, suggesting that the relationship between OHC electromotility and the peripheral sensitivity to sound may not be linear.
Review
Cell Biology
Yu-Bei Dai, Xiang Gao, Dong Liu, Jie Gong
Summary: Rho GTPases are crucial regulators of the actin cytoskeleton, involved in various physiological and biochemical processes. RhoA, Cdc42, and Rac1, members of the classical subfamily of Rho GTPases, play a role in the development and maintenance of cilia in cochlear hair cells. They also regulate the orientation of ciliary bundles and contribute to the motility of outer hair cells in the auditory sensory system. This review focuses on the expression of RhoA, Cdc42, and Rac1 in cochlear hair cells and their involvement in ciliary bundle morphogenesis and hair cell movement, as well as their potential as drug targets for deafness treatment.
NEURAL REGENERATION RESEARCH
(2023)
Article
Biochemistry & Molecular Biology
Yuwei Sun, Yu Zhang, Di Zhang, Guangqin Wang, Lei Song, Zhiyong Liu
Summary: Cochlear outer hair cells (OHCs) are important for hearing, and a specific enhancer for OHC damage treatment is currently unavailable. This study used CRISPR/Cas9 to delete large DNA fragments of Slc26a5 in mice and found that the deletion affected the expression of Prestin. Furthermore, the study identified a 1.4 kbp segment as an OHC-specific enhancer, which is likely to be evolutionarily conserved across different species.
Article
Cell Biology
Katharine K. Miller, Patrick Atkinson, Kyssia Ruth Mendoza, Daibhid Maoileidigh, Nicolas Grillet
Summary: The hair bundle of hair cells is a mechanosensory organelle that detects mechanical stimuli, and its dimensions and characteristics affect how it responds to stimuli. Measurements on mouse inner hair cells concluded that different fixation methods can influence the size and mechanical properties of hair bundles.
FRONTIERS IN CELL AND DEVELOPMENTAL BIOLOGY
(2021)
Article
Neurosciences
Jie Li, Shuang Liu, Chenmeng Song, Tong Zhu, Zhikai Zhao, Wenzhi Sun, Yi Wang, Lei Song, Wei Xiong
Summary: This study found that ultrahigh-frequency hearing in mice does not depend on the presence of Prestin protein, but rather on the OHCs. OHCs are still able to perform ultrahigh-frequency transduction without Prestin, and this process can be inhibited by a universal cation channel blocker, Gadolinium.
NEUROSCIENCE BULLETIN
(2022)
Article
Neurosciences
Marcelo J. Moglie, Diego L. Wengier, A. Belen Elgoyhen, Juan D. Goutman
Summary: Normal cochlear function requires precise control of intracellular Ca2+ levels, with Ca2+ influx occurring at the stereocilia tips and basolateral membrane. Two different sources of Ca2+ influx in the basolateral membrane, VGCCs and alpha 9 alpha 10 receptors, are regulated differently, suggesting well-tuned mechanisms to separate the two OHC synaptic functions.
JOURNAL OF NEUROSCIENCE
(2021)
Review
Audiology & Speech-Language Pathology
Wenxuan He, George Burwood, Anders Fridberger, Alfred L. Nuttall, Tianying Ren
Summary: This paper summarizes the experimental findings on the cochlear active process by the authors' group. The results show that outer hair cells are able to generate substantial forces for driving the cochlear partition at all audible frequencies in vivo, and the acoustically induced reticular lamina vibration is larger and more broadly tuned than the basilar membrane vibration. These findings provide new insights into our understanding of cochlear amplification mechanisms.
Article
Neurosciences
Snezana Levic, Victoria A. Lukashkina, Patricio Simoes, Andrei N. Lukashkin, Ian J. Russell
Summary: Cochlear amplification is achieved through the voltage-dependent electromotility of mechanosensory outer hair cells, which amplifies low-to-moderate level sounds and compresses loud sounds. This study investigates the role of receptor potentials and electrical properties of outer hair cells in high-frequency hearing. The results suggest that extracellular receptor potentials, rather than receptor potentials, drive outer hair cell motility and cochlear amplification at high frequencies.
JOURNAL OF NEUROSCIENCE
(2022)
Article
Neurosciences
Hong-Bo Zhao, Li-Man Liu, Ning Yu, Yan Zhu, Ling Mei, Jin Chen, Chun Liang
Summary: The study reveals a new pathway in the descending cochlear efferent system, where medial olivocochlear fibers innervate cochlear supporting cells to regulate their gap junctions, thus controlling outer hair cell electromotility and hearing sensitivity. This supporting cell gap junction-mediated efferent control pathway plays a crucial role in protecting hearing from noise.
JOURNAL OF NEUROPHYSIOLOGY
(2022)
Review
Neurosciences
Basile Tarchini
Summary: Sensory hair cells detect mechanical stimuli through their hair bundle structure, which determines their directional sensitivity. Coordinated orientation of same-side hair cells ensures the delivery of local group response, while the differential orientation of opposite-side hair cells provides bidirectional sensitivity at the organ level.
FRONTIERS IN NEUROSCIENCE
(2021)
Article
Acoustics
Renata Sisto, Arturo Moleti
Summary: The low-pass characteristic of OHC voltage response to mechanical stimulation may pose challenges for cochlear models, but evidence suggests that the local cutoff frequency changes approaching the cochlear base. By incorporating low-pass filtering for an internal force term, models can better simulate the frequency dependence of different regions of the cochlea.
JOURNAL OF THE ACOUSTICAL SOCIETY OF AMERICA
(2021)
Article
Audiology & Speech-Language Pathology
Patricia M. Quinones, Sebastiaan W. F. Meenderink, Brian E. Applegate, John S. Oghalai
Summary: The study found that unloaded OHC bundles do not spontaneously oscillate in the mouse cochlea, which is different from observations in non-mammalian ears.
Article
Neurosciences
Catherine J. C. Weisz, Sean-Paul G. Williams, Chad S. Eckard, Christopher B. Divito, David W. Ferreira, Kristen N. Fantetti, Shenin A. Dettwyler, Hou-Ming Cai, Maria E. Rubio, Karl Kandler, Rebecca P. Seal
Summary: The study reveals that cochlear outer hair cells, unlike inner hair cells, are capable of releasing glutamate and activating neurons in the cochlear nucleus at moderate sound levels.
JOURNAL OF NEUROSCIENCE
(2021)
Review
Genetics & Heredity
Nicolas Michalski, Christine Petit
Summary: Research on inherited hearing disorders has identified numerous causal genes and shown potential for gene therapy in mouse models. Understanding molecular mechanisms of cochlear development and/or physiology has been elucidated, but central auditory dysfunctions may also impact outcomes of peripheral gene therapy for hearing restoration.
Article
Biochemistry & Molecular Biology
Imane AitRaise, Ghita Amalou, Amale Bousfiha, Hicham Charoute, Hassan Rouba, Houria Abdelghaffar, Crystel Bonnet, Christine Petit, Adbelhamid Barakat
Summary: This study identified the genetic causes of deafness in six Moroccan families using whole exome sequencing and validated mutations in four genes. The results highlight the importance of whole exome sequencing in identifying pathogenic mutations in heterogeneous disorders with multiple genes responsible.
MOLECULAR BIOLOGY REPORTS
(2022)
Article
Clinical Neurology
Francoise Lazarini, Annie Lannuzel, Andre Cabie, Vincent Michel, Yoann Madec, Hugo Chaumont, Isabelle Calmont, Mathilde Favrat, Xavier Montagutelli, Emmanuel Roze, Pierre-Marie Lledo
Summary: ZIKV infection is associated with poorer olfactory function in patients with GBS and also causes hyposmia in infected mice. These findings suggest that ZIKV should be considered as a virus affecting the olfactory system, and clinical evaluation of the olfactory system is important for ZIKV-infected patients.
EUROPEAN JOURNAL OF NEUROLOGY
(2022)
Article
Biology
Celine Trebeau, Jacques Boutet de Monvel, Gizem Altay, Jean-Yves Tinevez, Raphael Etournay
Summary: Efficient tools for extracting 2D surfaces from 3D microscopy data are essential for studying the complex cellular choreography of epithelium morphogenesis. The Zellige software allows the extraction of multiple surfaces with varying inclination, contrast, and texture from a 3D image, providing a solution to the limitations of existing methods. The software has been demonstrated to perform well on synthetic images and various biological samples.
Article
Audiology & Speech-Language Pathology
Olivier Postal, Warren Bakay, Typhaine Dupont, Alexa Buck, Elodie Daoud, Christine Petit, Nicolas Michalski, Boris Gourevitch
Summary: Auditory Brainstem Responses (ABRs) are reliable for measuring auditory thresholds, but cannot detect changes in higher auditory areas. Cortical Auditory Evoked Potentials (CAEPs) capture information from these areas but are less frequently used as diagnostic tools due to their variability. This study developed a consistent measure of subcutaneous CAEPs in mice and determined their origin.
Editorial Material
Medicine, Research & Experimental
Paul Avan, Christine Petit
M S-MEDECINE SCIENCES
(2022)
Article
Multidisciplinary Sciences
Charlotte Calvet, Thibault Peineau, Najate Benamer, Maxence Cornille, Andrea Lelli, Baptiste Plion, Ghizlene Lahlou, Julia Fanchette, Sylvie Nouaille, Jacques Boutet de Monvel, Amrit Estivalet, Philippe Jean, Vincent Michel, Martin Sachse, Nicolas Michalski, Paul Avan, Christine Petit, Didier Dulon, Saaid Safieddine
Summary: This study found that SNAP-25 is essential for normal hearing function by ensuring exocytosis and maintenance of ribbon synapses in IHCs.
Article
Otorhinolaryngology
Malak Salame, Crystel Bonnet, Ely Cheikh Mohamed Moctar, Selma Mohamed Brahim, Abdallahi Dedy, Ledour Abdel Vetah, Fatimetou Veten, Cheikh Tijani Hamed, Christine Petit, Ahmed Houmeida
Summary: In this study, the exon 7 of the LRTOMT gene was screened in a cohort of congenital deaf children from Mauritania, and a novel pathogenic mutation was identified. The mutation was found to disrupt the structure of the encoded protein and early cochlear implant fitting seemed to improve the auditory ability of the mutation carrier. Further screening of deafness genes may reveal other variants underlying hearing impairment in the population.
EUROPEAN ARCHIVES OF OTO-RHINO-LARYNGOLOGY
(2023)
Review
Genetics & Heredity
Christine Petit, Crystel Bonnet, Saaid Safieddine
Summary: Progress in genetic studies of SNHI and multidisciplinary studies of mouse models have elucidated the molecular mechanisms underlying auditory system function, leading to the development of inner-ear gene therapy. Preclinical studies have highlighted key translational opportunities and challenges for treating monogenic forms of SNHI and associated balance disorders.
NATURE REVIEWS GENETICS
(2023)
Article
Multidisciplinary Sciences
Philippe Jean, Fabienne Wong Jun Tai, Amrit Singh-Estivalet, Andrea Lelli, Cyril Scandola, Sebastien Megharba, Sandrine Schmutz, Solene Roux, Sabrina Mechaussier, Muriel Sudres, Enguerran Mouly, Anne-Valerie Heritier, Crystel Bonnet, Adeline Mallet, Sophie Novault, Valentina Libri, Christine Petit, Nicolas Michalski
Summary: Functional molecular characterization of the cochlea has mainly been driven by the deciphering of the genetic architecture of sensorineural deafness. By analyzing the single-cell transcriptomic atlas of the mouse cochlea, researchers have identified almost all cochlear cell types and discovered three cell types, providing insights into the gene regulatory networks controlling cochlear cell differentiation and maturation.
PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA
(2023)
Article
Cell Biology
Batiste Boeda, Vincent Michel, Raphael Etournay, Patrick England, Stephane Rigaud, Heloise Mary, Samy Gobaa, Sandrine Etienne-Manneville
Summary: Using an organ-on-chip model, this study elucidates the role of SCRIB in controlling cell and tissue shape during gut epithelium differentiation. The results show that SCRIB acts as a scaffold for SHROOM2/4 and ROCK1, controlling myosin polarization and playing a critical role in apical cell shape and epithelial morphogenesis.
JOURNAL OF CELL BIOLOGY
(2023)
Article
Multidisciplinary Sciences
Nell Saunders, Ignacio Fernandez, Cyril Planchais, Vincent Michel, Maaran Michael Rajah, Eduard Baquero Salazar, Jeanne Postal, Francoise Porrot, Florence Guivel-Benhassine, Catherine Blanc, Gaelle Chauveau-Le Friec, Augustin Martin, Ludivine Grzelak, Rischa Maya Oktavia, Annalisa Meola, Olivia Ahouzi, Hunter Hoover-Watson, Matthieu Prot, Deborah Delaune, Marion Cornelissen, Martin Deijs, Veronique Meriaux, Hugo Mouquet, Etienne Simon-Loriere, Lia van der Hoek, Pierre Lafaye, Felix Rey, Julian Buchrieser, Olivier Schwartz
Summary: In this study, it was found that TMPRSS2 is a functional receptor for coronavirus HKU1, triggering cell-cell fusion and viral entry. Designed anti-TMPRSS2 nanobodies were shown to inhibit HKU1 spike attachment and infection.
Article
Ophthalmology
Vasily M. Smirnov, Marco Nassisi, Saddek Mohand-Said, Crystel Bonnet, Anne Aubois, Celine Devisme, Thilissa Dib, Christina Zeitz, Natalie Loundon, Sandrine Marlin, Christine Petit, Bahram Bodaghi, Jose-Alain Sahel, Isabelle Audo
Summary: This study provides a detailed phenotypic description of rod-cone dystrophy associated with CLRN1 variants. The retinal phenotype includes visual acuity loss, visual field constriction, and retinal degeneration. Four pathogenic variants were identified in the study.
INVESTIGATIVE OPHTHALMOLOGY & VISUAL SCIENCE
(2022)