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
Marco Manca, Piece Yen, Paolo Spaiardi, Giancarlo Russo, Roberta Giunta, Stuart L. Johnson, Walter Marcotti, Sergio Masetto
Summary: The residual I-Ca in both auditory and vestibular hair cells from Ca(V)1.3(-/-) mice was significantly reduced, but the K+ current profile changes in vestibular hair cells suggest that non-exocytotic transmission remains functional.
FRONTIERS IN NEUROSCIENCE
(2021)
Article
Multidisciplinary Sciences
Andrew A. Mecca, Giusy A. Caprara, Anthony W. Peng
Summary: This study found that up-regulation of cAMP lowers the sensitivity of the channel by decreasing the stiffness of the gating spring. Prolonged depolarization can also mimic the effects of cAMP. The results suggest that cAMP signaling modulates gating spring stiffness to affect auditory sensitivity.
PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA
(2022)
Article
Neurosciences
Alexandra L. Scharr, Daibhid O. Maoileidigh, Anthony J. Ricci
Summary: The hair bundle, composed of mechanically coupled stereocilia, is a universal mechanosensory organelle in auditory, vestibular, and lateral-line systems. The dynamics of individual stereocilia in response to bundle stimulus has not been quantified. By stimulating and tracking individual inner-hair-cell stereocilia, it was found that nonuniform stimulation led to dissimilar stereociliary displacements and stereocilia closer to the stimulator moved more. The weak coupling between stereocilia suggests that more uniform stimulation across the tallest stereocilia is required to enhance the receptor current.
JOURNAL OF NEUROSCIENCE
(2023)
Article
Neurosciences
Giusy A. Caprara, Anthony W. Peng
Summary: The auditory and vestibular systems in the inner ear detect and translate sensory information related to sound and balance. Hair cells, which are sensory cells, transform mechanical input into electrical signals, with the specialized organelle called the hair bundle detecting mechanical signals. Different types of hair cells in the auditory and vestibular systems have distinct morphology and characteristics in the mechanotransduction process, contributing to the efficient transduction of specific signals in each system.
MOLECULAR AND CELLULAR NEUROSCIENCE
(2022)
Article
Multidisciplinary Sciences
Tianli Qin, Chin Chung Ho, Boshi Wang, Chi-Chung Hui, Mai Har Sham
Summary: This study reveals that Sufu and Spop play crucial roles in controlling the timing and progression of sensory cell differentiation in the development of mammalian auditory epithelium. They maintain the progenitor state of sensory progenitor cells through regulation of the Shh-Gli2 signaling pathway, thus preventing hair cell differentiation.
PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA
(2022)
Review
Cell Biology
Difei Wang, Jun Zhou
Summary: Primary cilia are highly specialized organelles protruding from cell membranes, and mutations in genes encoding ciliary proteins can cause ciliopathies; this paper reviews the structure and function of specialized primary cilia, known as kinocilia, in the mammalian auditory system, and discusses potential therapeutic approaches for auditory ciliopathies.
FRONTIERS IN CELL AND DEVELOPMENTAL BIOLOGY
(2021)
Article
Multidisciplinary Sciences
Xiaofen Li, Qirui Zhao, Xiaojie Yu, Wenhan Cao, Yingyi Zhang, Wanying Feng, Liwen Jiang, David Z. He, Robert Z. Qi, Pingbo Huang
Summary: In this study, a previously undescribed organelle called apicosome was identified in hair cells of the neonatal mouse inner ear. It has a diameter of approximately 500 nm and shows transient appearance and itinerant nature during cochlear hair cell development. Unlike cochlear hair cells, the apicosome persists in vestibular hair cells even in adults. The timing of apicosome translocation and disappearance in cochlear hair cells during development is correlated with kinocilium development and maintenance. Interestingly, transdifferentiated hair cells from supporting cells also contain apicosome.
Article
Multidisciplinary Sciences
Jie Li, Shuang Liu, Chenmeng Song, Qun Hu, Zhikai Zhao, Tuantuan Deng, Yi Wang, Tong Zhu, Linzhi Zou, Shufeng Wang, Jiaofeng Chen, Lian Liu, Hanqing Hou, Kexin Yuan, Hairong Zheng, Zhiyong Liu, Xiaowei Chen, Wenzhi Sun, Bailong Xiao, Wei Xiong
Summary: The knockout of the mechanosensitive ion channel PIEZO2 in cochlea disrupts ultrasonic hearing in mice, but not low-frequency hearing. Deletion of Piezo2 in outer hair cells specifically abolishes associative learning in mice during hearing exposure at ultrasonic frequencies. The study demonstrates that OHCs serve as effector cells, combining with PIEZO2 as an essential molecule for ultrasonic hearing in mice.
PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA
(2021)
Article
Cell Biology
Keji Yan, Wen Zong, Haibo Du, Xiaoyan Zhai, Rui Ren, Shuang Liu, Wei Xiong, Yanfei Wang, Zhigang Xu
Summary: In this study, it was found that BAIAP2L2 is localized at the tips of inner ear hair cells' stereocilia, and its inactivation leads to degeneration of mechanotransducing stereocilia, resulting in hearing loss. BAIAP2L2 binds to components of the row 2 complex and MET complex, indicating its crucial role in maintaining stereocilia.
JOURNAL OF CELLULAR PHYSIOLOGY
(2022)
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
Neurosciences
Yang Yang, Kaitlin Murtha, Leslie K. Climer, Federico Ceriani, Pierce Thompson, Aubrey J. Hornak, Walter Marcotti, Dwayne D. Simmons
Summary: Outer hair cells (OHCs) play a crucial role in the frequency selectivity and sensitivity of mammalian hearing. This study shows that the calcium-binding protein oncomodulin (OCM) regulates the spontaneous Ca2+ activity and afferent connectivity of OHCs during development.
JOURNAL OF PHYSIOLOGY-LONDON
(2023)
Article
Oncology
Dan You, Jin Guo, Yunzhong Zhang, Luo Guo, Xiaoling Lu, Xinsheng Huang, Shan Sun, Huawei Li
Summary: Using single-cell sequencing, this study found that new hair cells in the utricle mainly arise from differentiation of Sox9+ progenitor cells, with only a small number of cells being generated by mitotic proliferation. Mitotically generated hair cells were primarily found in the extrastriola in adult mice. The study also demonstrated that both Type II and Type I hair cells can be regenerated through either mitotic cell proliferation or progenitor cell differentiation.
CLINICAL AND TRANSLATIONAL MEDICINE
(2022)
Article
Chemistry, Multidisciplinary
Xiaolong Fu, Peifeng Wan, Ling Lu, Yingcui Wan, Ziyi Liu, Guodong Hong, Shengda Cao, Xiuli Bi, Jing Zhou, Ruifeng Qiao, Siwei Guo, Yu Xiao, Bingzheng Wang, Miao Chang, Wen Li, Peipei Li, Aizhen Zhang, Jin Sun, Renjie Chai, Jiangang Gao
Summary: In this study, two peroxisome-deficient mouse models were established to investigate the role of peroxisomes in hearing. The results showed that peroxisomes mainly function in the hair cells of cochleae and regulate BK channels, which are essential for auditory function. These findings suggest that BK channels could be potential targets for treating peroxisome-related hearing diseases.
Article
Neurosciences
Ting Fan, Meng-Ya Xiang, Ruo-Qiao Zhou, Wen Li, Li-Qin Wang, Peng-Fei Guan, Geng-Lin Li, Yun-Feng Wang, Jian Li
Summary: The study found that high concentrations of sodium salicylate can cause significant damage to inner hair cells in the cochlea of mice and increase the number of Ca2+ channels by altering Ca2+ current and expression of Ca(V)1.3, leading to enhanced Ca2+ influx into IHCs, which may contribute to tinnitus generation in the peripheral auditory system.
NEUROSCIENCE BULLETIN
(2022)
Article
Biophysics
Srdjan Prodanovic, Sheryl Gracewski, Jong-Hoon Nam
BIOPHYSICAL JOURNAL
(2015)
Article
Biophysics
Jong-Hoon Nam, Anthony W. Peng, Anthony J. Ricci
BIOPHYSICAL JOURNAL
(2015)
Article
Biochemistry & Molecular Biology
Sedigheh Delmaghani, Jean Defourny, Asadollah Aghaie, Maryline Beurg, Didier Dulon, Nicolas Thelen, Isabelle Perfettini, Tibor Zelles, Mate Aller, Anais Meyer, Alice Emptoz, Fabrice Giraudet, Michel Leibovici, Sylvie Dartevelle, Guillaume Soubigou, Marc Thiry, E. Sylvester Vizi, Saaid Safieddine, Jean-Pierre Hardelin, Paul Avan, Christine Petit
Article
Physiology
Maryline Beurg, Adam C. Goldring, Robert Fettiplace
JOURNAL OF GENERAL PHYSIOLOGY
(2015)
Article
Neurosciences
Zizhen Wu, Nicolas Grillet, Bo Zhao, Christopher Cunningham, Sarah Harkins-Perry, Bertrand Coste, Sanjeev Ranade, Navid Zebarjadi, Maryline Beurg, Robert Fettiplace, Ardem Patapoutian, Ulrich Muller
NATURE NEUROSCIENCE
(2017)
Article
Multidisciplinary Sciences
Maryline Beurg, Adam C. Goldring, Anthony J. Ricci, Robert Fettiplace
PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA
(2016)
Review
Neurosciences
Maryline Beurg, Robert Fettiplace
JOURNAL OF PHYSIOLOGY-LONDON
(2017)
Article
Biochemical Research Methods
Yanju Liu, Sheryl M. Gracewski, Jong-Hoon Nam
PLOS COMPUTATIONAL BIOLOGY
(2017)
Article
Acoustics
Daniel Marnell, Talat Jabeen, Jong-Hoon Nam
JOURNAL OF THE ACOUSTICAL SOCIETY OF AMERICA
(2018)
Article
Multidisciplinary Sciences
Maryline Beurg, Wei Xiong, Bo Zhao, Ulrich Mueller, Robert Fettiplace
PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA
(2015)
Review
Audiology & Speech-Language Pathology
Robert Fettiplace, Jong-Noon Nam
Article
Biophysics
Talat Jabeen, Joseph C. Holt, Jonathan R. Becker, Jong-Hoon Nam
BIOPHYSICAL JOURNAL
(2020)
Article
Neurosciences
Wenxiao Zhou, Talat Jabeen, Sultan Sabha, Jonathan Becker, Jong-Hoon Nam
Summary: This article focuses on the mechanical properties of outer hair cells and the surrounding structures in the mammalian cochlea, as well as their impact on mammalian hearing. The study finds that the stiffness of the tectorial membrane and the Deiters cell play a critical role in determining the relative motion among different structures in the organ of Corti. The Deiters cells act as a mechanical equalizer to ensure that the outer hair cells are neither too rigid nor too weak.
JOURNAL OF NEUROSCIENCE
(2022)
Article
Multidisciplinary Sciences
Wenxiao Zhou, Jong-Hoon Nam
SCIENTIFIC REPORTS
(2019)
Proceedings Paper
Biochemistry & Molecular Biology
Jong-Hoon Nam, Yanju Liu, Sheryle M. Gracewski
MECHANICS OF HEARING: PROTEIN TO PERCEPTION
(2015)