PMID- 31827424
OWN - NLM
STAT- PubMed-not-MEDLINE
LR  - 20240422
IS  - 1662-5102 (Print)
IS  - 1662-5102 (Electronic)
IS  - 1662-5102 (Linking)
VI  - 13
DP  - 2019
TI  - Structure and Function of Cochlear Gap Junctions and Implications for the 
      Translation of Cochlear Gene Therapies.
PG  - 529
LID - 10.3389/fncel.2019.00529 [doi]
LID - 529
AB  - 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 innervation of nerve fibers to the hair cells (HCs), the maturation of the 
      ribbon synapses, and the initiation of the endocochlear potential (EP). New data, 
      especially those collected from targeted modification of major Cx genes in the 
      mouse cochlea, have demonstrated that Cx26 plays an essential role in the 
      postnatal maturation of the cochlea. Studies also show that Cx26 and Cx30 assume 
      very different roles in the EP generation, given that only Cx26 is required for 
      normal hearing. This article will review our current understanding of the 
      molecular structure, cellular distribution, and major functions of cochlear GJs. 
      Potential implications of the knowledge of cochlear GJs on the design and 
      implementation of translational studies of cochlear gene therapies for Cx 
      mutations are also discussed.
CI  - Copyright (c) 2019 Wu, Zhang, Li and Lin.
FAU - Wu, Xuewen
AU  - Wu X
AD  - Department of Otolaryngology, Head-Neck and Surgery, Xiangya Hospital of Central 
      South University, Changsha, China.
AD  - Department of Otolaryngology, Emory University School of Medicine, Atlanta, GA, 
      United States.
FAU - Zhang, Wenjuan
AU  - Zhang W
AD  - Department of Otolaryngology, Wuhan Union Hospital, Tongji Medical College, 
      Huazhong University of Science and Technology, Wuhan, China.
FAU - Li, Yihui
AU  - Li Y
AD  - Department of Pharmacy, Changsha Hospital of Traditional Medicine, Changsha, 
      China.
FAU - Lin, Xi
AU  - Lin X
AD  - Department of Otolaryngology, Emory University School of Medicine, Atlanta, GA, 
      United States.
LA  - eng
GR  - R01 DC006483/DC/NIDCD NIH HHS/United States
GR  - R01 DC014496/DC/NIDCD NIH HHS/United States
PT  - Journal Article
DEP - 20191127
PL  - Switzerland
TA  - Front Cell Neurosci
JT  - Frontiers in cellular neuroscience
JID - 101477935
PMC - PMC6892400
OTO - NOTNLM
OT  - cochlea
OT  - connexin
OT  - deafness
OT  - gap junction
OT  - gene therapy
OT  - structure and function
EDAT- 2019/12/13 06:00
MHDA- 2019/12/13 06:01
PMCR- 2019/01/01
CRDT- 2019/12/13 06:00
PHST- 2019/09/18 00:00 [received]
PHST- 2019/11/13 00:00 [accepted]
PHST- 2019/12/13 06:00 [entrez]
PHST- 2019/12/13 06:00 [pubmed]
PHST- 2019/12/13 06:01 [medline]
PHST- 2019/01/01 00:00 [pmc-release]
AID - 10.3389/fncel.2019.00529 [doi]
PST - epublish
SO  - Front Cell Neurosci. 2019 Nov 27;13:529. doi: 10.3389/fncel.2019.00529. 
      eCollection 2019.