Connexin-mediated signaling in nonsensory cells is crucial for the development of sensory inner hair cells in the mouse cochlea
SL Johnson, F Ceriani, O Houston… - Journal of …, 2017 - Soc Neuroscience
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 …
30 (Cx30) have been linked to syndromic and nonsyndromic hearing loss in mice and …
Inner ear connexin channels: roles in development and maintenance of cochlear function
F Mammano - Cold Spring Harbor perspectives …, 2019 - perspectivesinmedicine.cshlp.org
Connexin 26 and connexin 30 are the prevailing isoforms in the epithelial and connective
tissue gap junction systems of the developing and mature cochlea. The most frequently …
tissue gap junction systems of the developing and mature cochlea. The most frequently …
Progressive age-dependence and frequency difference in the effect of gap junctions on active cochlear amplification and hearing
L Zong, J Chen, Y Zhu, HB Zhao - Biochemical and biophysical research …, 2017 - Elsevier
Abstract Mutations of Connexin 26 (Cx26, GJB2), which is a predominant gap junction
isoform in the cochlea, can induce high incidence of nonsyndromic hearing loss. We …
isoform in the cochlea, can induce high incidence of nonsyndromic hearing loss. We …
Gap junction-mediated intercellular biochemical coupling in cochlear supporting cells is required for normal cochlear functions
Y Zhang, W Tang, S Ahmad, JA Sipp… - Proceedings of the …, 2005 - National Acad Sciences
Dysfunction of gap junctions (GJs) caused by mutations in connexin26 (Cx26) and Cx30
accounts for nearly half of all cases of hereditary nonsyndromic deafness cases. Although it …
accounts for nearly half of all cases of hereditary nonsyndromic deafness cases. Although it …
A connexin30 mutation rescues hearing and reveals roles for gap junctions in cochlear amplification and micromechanics
VA Lukashkina, S Levic, AN Lukashkin… - Nature …, 2017 - nature.com
Accelerated age-related hearing loss disrupts high-frequency hearing in inbred CD-1 mice.
The p. Ala88Val (A88V) mutation in the gene coding for the gap-junction protein connexin30 …
The p. Ala88Val (A88V) mutation in the gene coding for the gap-junction protein connexin30 …
A gap-junction mutation reveals that outer hair cell extracellular receptor potentials drive high-frequency cochlear amplification
Cochlear amplification enables the enormous dynamic range of hearing through amplifying
cochlear responses to low-to moderate-level sounds and compressing them to loud sounds …
cochlear responses to low-to moderate-level sounds and compressing them to loud sounds …
Cochlear outer hair cells in a dominant-negative connexin26 mutant mouse preserve non-linear capacitance in spite of impaired distortion product otoacoustic …
A Minekawa, T Abe, A Inoshita, T Iizuka, S Kakehata… - Neuroscience, 2009 - Elsevier
Mutations in the connexin26 gene (GJB2) are the most common genetic cause of congenital
bilateral non-syndromic sensorineural hearing loss. Transgenic mice were established …
bilateral non-syndromic sensorineural hearing loss. Transgenic mice were established …
Preservation of developmental spontaneous activity enables early auditory system maturation in deaf mice
Intrinsically generated neural activity propagates through the developing auditory system to
promote maturation and refinement of sound processing circuits prior to hearing onset. This …
promote maturation and refinement of sound processing circuits prior to hearing onset. This …
Connexin30 null and conditional connexin26 null mice display distinct pattern and time course of cellular degeneration in the cochlea
Y Sun, W Tang, Q Chang, Y Wang… - Journal of …, 2009 - Wiley Online Library
Abstract Mutations in connexin26 (Cx26) and Cx30 are the most common cause of
nonsyndromic inherited deafness in humans. To understand the underlying molecular …
nonsyndromic inherited deafness in humans. To understand the underlying molecular …
Analysis of connexin subunits required for the survival of vestibular hair cells
YAN Qu, W Tang, I Dahlke, D Ding… - Journal of …, 2007 - Wiley Online Library
Mutations in connexin (Cx) genes are responsible for a large proportion of human inherited
prelingual deafness cases. The most commonly found human Cx mutations are either Cx26 …
prelingual deafness cases. The most commonly found human Cx mutations are either Cx26 …