Article (Scientific journals)
Recent insights into gap junction biogenesis in the cochlea
Defourny, Jean; Thiry, Marc
2022In Developmental Dynamics
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Keywords :
Cochlea; Connexin 26; Connexin 30; Gap junctions; GJB2; GJB6; Hearing loss
Abstract :
[en] 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.
Disciplines :
Biochemistry, biophysics & molecular biology
Author, co-author :
Defourny, Jean  ;  Université de Liège - ULiège > GIGA > GIGA Neurosciences - Cellular and tissular biology
Thiry, Marc  ;  Université de Liège - ULiège > Département des sciences de la vie > Service collectif des enseignements de biologie en bachelier ; Université de Liège - ULiège > Département de Biologie, Ecologie et Evolution > Collectif des enseignements de biologie en bachelier ; Université de Liège - ULiège > GIGA > GIGA Neurosciences - Cellular and tissular biology ; Université de Liège - ULiège > Département des sciences de la vie > Biologie cellulaire
Language :
English
Title :
Recent insights into gap junction biogenesis in the cochlea
Publication date :
26 September 2022
Journal title :
Developmental Dynamics
ISSN :
1058-8388
eISSN :
1097-0177
Publisher :
John Wiley & Sons, Hoboken, United States - New York
Peer reviewed :
Peer Reviewed verified by ORBi
Available on ORBi :
since 28 September 2022

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