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 …
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 …
GJB2 gene therapy and conditional deletion reveal developmental stage-dependent effects on inner ear structure and function
J Guo, X Ma, JM Skidmore, J Cimerman… - … Therapy-Methods & …, 2021 - cell.com
Pathogenic variants in GJB2, the gene encoding connexin 26, are the most common cause
of autosomal-recessive hereditary deafness. Despite this high prevalence, pathogenic …
of autosomal-recessive hereditary deafness. Despite this high prevalence, pathogenic …
Timed conditional null of connexin26 in mice reveals temporary requirements of connexin26 in key cochlear developmental events before the onset of hearing
Q Chang, W Tang, Y Kim, X Lin - Neurobiology of disease, 2015 - Elsevier
Mutations in the Gjb2 gene, which encodes a gap junction protein connexin26 (Cx26), are
the most prevalent form of hereditary deafness in humans and represent about half of non …
the most prevalent form of hereditary deafness in humans and represent about half of non …
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 …
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 …
Postnatal development of the organ of Corti in dominant-negative Gjb2 transgenic mice
A Inoshita, T Iizuka, HO Okamura, A Minekawa… - Neuroscience, 2008 - Elsevier
Hereditary hearing loss is one of the most prevalent inherited human birth defects, affecting
one in 2000. A strikingly high proportion (50%) of congenital bilateral nonsyndromic …
one in 2000. A strikingly high proportion (50%) of congenital bilateral nonsyndromic …
Homeostatic control of spontaneous activity in the developing auditory system
Neurons in the developing auditory system exhibit spontaneous bursts of activity before
hearing onset. How this intrinsically generated activity influences development remains …
hearing onset. How this intrinsically generated activity influences development remains …
Virally expressed connexin26 restores gap junction function in the cochlea of conditional Gjb2 knockout mice
Q Yu, Y Wang, Q Chang, J Wang, S Gong, H Li, X Lin - Gene therapy, 2014 - nature.com
Mutations in GJB2, which codes for the gap junction (GJ) protein connexin26 (Cx26), are the
most common causes of human nonsyndromic hereditary deafness. We inoculated modified …
most common causes of human nonsyndromic hereditary deafness. We inoculated modified …
Developmental abnormalities in supporting cell phalangeal processes and cytoskeleton in the Gjb2 knockdown mouse model
S Chen, L Xie, K Xu, HY Cao, X Wu… - Disease models & …, 2018 - journals.biologists.com
Mutations in the GJB2 gene [which encodes connexin 26 (Cx26)] are the most common
causes of hereditary hearing loss in humans, and previous studies showed postnatal …
causes of hereditary hearing loss in humans, and previous studies showed postnatal …