Results 71 to 80 of about 91,097 (243)

Connexin 26 Functions as a Direct Transcriptional Regulator During the Cochlea Development

Advanced Science, EarlyView.
Connexin26 can not only form intercellular channels that mediate rapid communication on the cell membrane, but also enter the nucleus as a transcription factor to directly regulate the transcription of nuclear genes. In the developing cochlea, Cx26 can control the maturation of the molecular scissor ADAM10 by regulating the transcription of TspanC8 ...
Xiaozhou Liu, Le Xie, Yuan Jin, Sen Chen, Xinyu Shi, Yanjun Zong, Zhengdong Zhao, Wei‐Jia Kong, Yu Sun   +8 more
wiley   +1 more source

Dual AAV gene therapy achieves recovery of hearing and auditory processing in a DFNB16 mouse model. [PDF]

Clin Transl Med
DFNB16 is among the most prevalent forms of congenital deafness, caused by mutations in the Stereocilin gene. Although no treatment currently exists, gene therapy represents a promising curative approach. Here, we demonstrate that AAV‐mediated gene delivery in a DFNB16 mouse model restored both peripheral hearing and central auditory processing ...
Iranfar S, Bagur S, Felgerolle C, Cornille M, Benamer N, Ribeuz HL, Giorgi M, Jamali S, Saoudi A, Lecomte MJ, Michel V, Bathellier B, Safieddine S.   +12 more
europepmc   +2 more sources

Connexin-Mediated Signaling in Nonsensory Cells Is Crucial for the Development of Sensory Inner Hair Cells in the Mouse Cochlea

Journal of Neuroscience, 2017
Mutations in the genes encoding for gap junction proteins connexin 26 (Cx26) and connexin 30 (Cx30) have been linked to syndromic and nonsyndromic hearing loss in mice and humans. The release of ATP from connexin hemichannels in cochlear nonsensory cells
Stuart L. Johnson, F. Ceriani, Oliver Houston, R. Polishchuk, E. Polishchuk, G. Crispino, Veronica Zorzi, F. Mammano, W. Marcotti   +8 more
semanticscholar   +1 more source

Cidea Targeting Protects Cochlear Hair Cells and Hearing Function From Drug‐ and Noise‐Induced Damage

Advanced Science, EarlyView.
Acquired sensorineural hearing loss is primarily caused by the damage or loss of cochlear hair cells, induced by factors such as noise exposure and ototoxic drugs. The deficiency of apoptosis‐inducing gene Cidea in Cidea KO mice or by delivering CRISPR/SlugCas9‐HF via AAV to edit Cidea effectively alleviated hair cell damage and hearing loss caused by ...
Shasha Zhang, Ruiying Qiang, Yuan Zhang, Jinxian Wan, Chen Tao, Ying Dong, Xujun Tang, Li Xu, Hairong Xiao, Yanqin Lin, Wei Tong, Ying Ma, Yongming Wang, Peng Li, Renjie Chai   +14 more
wiley   +1 more source

Traceless Regulation of Genetic Circuitry

Advanced Science, EarlyView.
Energy‐based, as opposed to molecular, control offers unprecedented improvements in key circuit parameters. This review summarizes the fundamentals of such traceless switches, categorizes them by trigger modalities, and compares and contrasts distinct advantages as well as shortcomings of each kind.
Gokberk Unal, Martin Fussenegger
wiley   +1 more source

RNA-seq transcriptomic analysis of adult zebrafish inner ear hair cells

Scientific Data, 2018
Although hair cells are the sensory receptors of the auditory and vestibular systems in the ears of all vertebrates, hair cell properties are different between non-mammalian vertebrates and mammals.
C. Barta, Huizhan Liu, Lei Chen, Kimberlee P. Giffen, Yi Li, K. L. Kramer, K. Beisel, D. He   +7 more
semanticscholar   +1 more source

Hearing Loss: From Basic to Clinical Science

Advanced Science, EarlyView.
Abstract Hearing loss (HL) affects over 1.5 billion people globally, with genetic factors accounting for ≈50% of congenital cases. Therefore, HL has become a global health issue, driving extensive research from basic science to clinical applications. This Special Collection includes a total of 31 papers, among which 9 are review papers, 21 are research
Renjie Chai, Hai Huang, Jing Zou
wiley   +1 more source

Spiking Pattern of the Mouse Developing Inner Hair Cells Is Mostly Invariant Along the Tonotopic Axis

Frontiers in Cellular Neuroscience, 2018
During development, the sensory cells of the cochlea, the inner hair cells (IHCs), fire spontaneous calcium action potentials. This activity at the pre-hearing stage allows the IHCs to autonomously excite the auditory nerve fibers and hence, represents ...
Anne-Gabrielle Harrus, Jean-Charles Ceccato, Gaston Sendin, Jérôme Bourien, Jean-Luc Puel, Régis Nouvian   +5 more
doaj   +1 more source

Inner Hair Cell Loss Disrupts Hearing and Cochlear Function Leading to Sensory Deprivation and Enhanced Central Auditory Gain

Frontiers in Neuroscience, 2017
There are three times as many outer hair cells (OHC) as inner hair cells (IHC), yet IHC transmit virtually all acoustic information to the brain as they synapse with 90–95% of type I auditory nerve fibers.
R. Salvi, Wei Sun, D. Ding, Guang-di Chen, E. Lobarinas, Jian Wang, Kelly E. Radziwon, Benjamin D. Auerbach   +7 more
semanticscholar   +1 more source

Compensatory Interplay Between Clarin‐1 and Clarin‐2 Deafness‐Associated Proteins Governs Phenotypic Variability in Hearing

Advanced Science, EarlyView.
Functional compensation between clarin‐1 and clarin‐2 in cochlear hair cells. Hearing loss associated with CLRN1 mutations shows striking phenotypic variability; however, the underlying mechanisms remain poorly understood. This study reveals that clarin‐1 and clarin‐2 function cooperatively in cochlear hair cells to sustain mechanoelectrical ...
Maureen Wentling, Aïda Yakhlef Sanchez, Nicolas Thelen, Müge Senarisoy, Maria Hogg, Steven Condamine, Andrea Lelli, Emilia Wysocka, Pranav Patni, Sandrine Vitry, Kerem Yasin Yildizhan, Sébastien Le Gal, Sylvie Nouaille, Michael R. Bowl, Marc Thiry, Didier Dulon, Sedigheh Delmaghani, Aziz El‐Amraoui   +17 more
wiley   +1 more source