Preservation of developmental spontaneous activity enables early auditory system maturation in deaf mice

CJ Kersbergen, TA Babola, PO Kanold… - PLoS biology, 2023 - journals.plos.org
Intrinsically generated neural activity propagates through the developing auditory system to
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 …

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 …

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 …

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 …

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 …

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 …

Homeostatic control of spontaneous activity in the developing auditory system

TA Babola, S Li, A Gribizis, BJ Lee, JB Issa, HC Wang… - Neuron, 2018 - cell.com
Neurons in the developing auditory system exhibit spontaneous bursts of activity before
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 …

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 …