Recent insights into gap junction biogenesis in the cochlea.
GJB2
GJB6
cochlea
connexin 26
connexin 30
gap junctions
hearing loss
Journal
Developmental dynamics : an official publication of the American Association of Anatomists
ISSN: 1097-0177
Titre abrégé: Dev Dyn
Pays: United States
ID NLM: 9201927
Informations de publication
Date de publication:
02 2023
02 2023
Historique:
revised:
08
09
2022
received:
17
05
2022
accepted:
11
09
2022
pubmed:
16
9
2022
medline:
15
2
2023
entrez:
15
9
2022
Statut:
ppublish
Résumé
In the cochlea, connexin 26 (Cx26) and connexin 30 (Cx30) co-assemble into two types of homomeric and heteromeric gap junctions between adjacent non-sensory epithelial cells. These channels provide a mechanical coupling between connected cells, and their activity is critical to maintain cochlear homeostasis. Many of the mutations in GJB2 or GJB6, which encode Cx26 and Cx30 in humans, impair the formation of membrane channels and cause autosomal syndromic and non-syndromic hearing loss. Thus, deciphering the connexin trafficking pathways in situ should represent a major step forward in understanding the pathogenic significance of many of these mutations. A growing body of evidence now suggests that Cx26/Cx30 heteromeric and Cx30 homomeric channels display distinct assembly mechanisms. Here, we review the most recent advances that have been made toward unraveling the biogenesis and stability of these gap junctions in the cochlea.
Substances chimiques
Connexins
0
Connexin 30
0
Types de publication
Review
Journal Article
Research Support, Non-U.S. Gov't
Langues
eng
Sous-ensembles de citation
IM
Pagination
239-246Informations de copyright
© 2022 American Association for Anatomy.
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