Details
| Original language | English |
|---|---|
| Pages (from-to) | 177-86 |
| Number of pages | 10 |
| Journal | The journal of membrane biology |
| Volume | 209 |
| Issue number | 2-3 |
| Publication status | Published - 15 Jun 2006 |
Abstract
Gap junctions play a critical role in hearing and mutations in connexin genes cause a high incidence of human deafness. Pathogenesis mainly occurs in the cochlea, where gap junctions form extensive networks between non-sensory cells that can be divided into two independent gap junction systems, the epithelial cell gap junction system and the connective tissue cell gap junction system. At least four different connexins have been reported to be present in the mammalian inner ear, and gap junctions are thought to provide a route for recycling potassium ions that pass through the sensory cells during the mechanosensory transduction process back to the endolymph. Here we review the cochlear gap junction networks and their hypothesized role in potassium ion recycling mechanism, pharmacological and physiological gating of cochlear connexins, animal models harboring connexin mutations and functional studies of mutant channels that cause human deafness. These studies elucidate gap junction functions in the cochlea and also provide insight for understanding the pathogenesis of this common hereditary deafness induced by connexin mutations.
Keywords
- Animals, Cochlea/metabolism, Connexins/genetics, Disease Models, Animal, Gap Junctions/metabolism, Hearing Loss/genetics, Homeostasis/genetics, Humans, Mice, Mutation
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In: The journal of membrane biology, Vol. 209, No. 2-3, 15.06.2006, p. 177-86.
Research output: Contribution to journal › Review article › Research › peer review
}
TY - JOUR
T1 - Gap junctions and cochlear homeostasis
AU - Zhao, H-B
AU - Kikuchi, T
AU - Ngezahayo, A
AU - White, T W
PY - 2006/6/15
Y1 - 2006/6/15
N2 - Gap junctions play a critical role in hearing and mutations in connexin genes cause a high incidence of human deafness. Pathogenesis mainly occurs in the cochlea, where gap junctions form extensive networks between non-sensory cells that can be divided into two independent gap junction systems, the epithelial cell gap junction system and the connective tissue cell gap junction system. At least four different connexins have been reported to be present in the mammalian inner ear, and gap junctions are thought to provide a route for recycling potassium ions that pass through the sensory cells during the mechanosensory transduction process back to the endolymph. Here we review the cochlear gap junction networks and their hypothesized role in potassium ion recycling mechanism, pharmacological and physiological gating of cochlear connexins, animal models harboring connexin mutations and functional studies of mutant channels that cause human deafness. These studies elucidate gap junction functions in the cochlea and also provide insight for understanding the pathogenesis of this common hereditary deafness induced by connexin mutations.
AB - Gap junctions play a critical role in hearing and mutations in connexin genes cause a high incidence of human deafness. Pathogenesis mainly occurs in the cochlea, where gap junctions form extensive networks between non-sensory cells that can be divided into two independent gap junction systems, the epithelial cell gap junction system and the connective tissue cell gap junction system. At least four different connexins have been reported to be present in the mammalian inner ear, and gap junctions are thought to provide a route for recycling potassium ions that pass through the sensory cells during the mechanosensory transduction process back to the endolymph. Here we review the cochlear gap junction networks and their hypothesized role in potassium ion recycling mechanism, pharmacological and physiological gating of cochlear connexins, animal models harboring connexin mutations and functional studies of mutant channels that cause human deafness. These studies elucidate gap junction functions in the cochlea and also provide insight for understanding the pathogenesis of this common hereditary deafness induced by connexin mutations.
KW - Animals
KW - Cochlea/metabolism
KW - Connexins/genetics
KW - Disease Models, Animal
KW - Gap Junctions/metabolism
KW - Hearing Loss/genetics
KW - Homeostasis/genetics
KW - Humans
KW - Mice
KW - Mutation
U2 - 10.1007/s00232-005-0832-x
DO - 10.1007/s00232-005-0832-x
M3 - Review article
C2 - 16773501
VL - 209
SP - 177
EP - 186
JO - The journal of membrane biology
JF - The journal of membrane biology
SN - 0022-2631
IS - 2-3
ER -