Structure and Function of Cochlear Gap Junctions and Implications for the Translation of Cochlear Gene Therapies.

ABSTRACT: Connexins (Cxs) are ubiquitous membrane proteins that are found throughout vertebrate organs, acting as building blocks of the gap junctions (GJs) known to play vital roles in the normal function of many organs. Mutations in Cx genes (particularly GJB2, which encodes Cx26) cause approximately half of all cases of congenital hearing loss in newborns. Great progress has been made in understanding GJ function and the molecular mechanisms for the role of Cxs in the cochlea. Data reveal that multiple types of Cxs work together to ensure normal development and function of the cochlea. These findings include many aspects not proposed in the classic K+ recycling theory, such as the formation of normal cochlear morphology (e.g., the opening of the tunnel of Corti), the fine-tuning of the innervatio

SUBMITTER: Wu X 

PROVIDER: S-EPMC6892400 | biostudies-literature | 2019

REPOSITORIES: biostudies-literature