PMID- 35345836
OWN - NLM
STAT- PubMed-not-MEDLINE
LR  - 20220330
IS  - 1662-5099 (Print)
IS  - 1662-5099 (Electronic)
IS  - 1662-5099 (Linking)
VI  - 14
DP  - 2021
TI  - F-Actin Dysplasia Involved in Organ of Corti Deformity in Gjb2 Knockdown Mouse 
      Model.
PG  - 808553
LID - 10.3389/fnmol.2021.808553 [doi]
LID - 808553
AB  - Mutations in the GJB2 gene encoding connexin26 (Cx26) protein are one of the most 
      common causes of hereditary deafness. Previous studies have found that different 
      Cx26-null mouse models have severe hearing loss and deformity of the organ of 
      Corti (OC) as well as a reduction in microtubules in pillar cells (PCs). To 
      explore the underlying mechanism of OC deformity caused by Cx26 downregulation 
      further, we established Cx26 knockdown (KD) mouse models at postnatal days (P)0 
      and P8. The actin filaments contained in the pillar cells of mice in the P0 KD 
      group were reduced by 54.85% and vinculin was increased by 22%, while the outer 
      hair cells (OHCs) showed normal F-actin content. In the P8 KD group, PCs and OHCs 
      of mice also showed almost normal F-actin content. The G-actin/F-actin ratio 
      increased by 38% in the P0 KD group. No significant change was found in the mRNA 
      or protein expression level of G-actin or the cadherin-catenin core complex in 
      the P0 KD group at P6. Moreover, immunofluorescence showed that the intensity of 
      LRRK2 was reduced by 97% in the P0 KD group at P6. Our results indicate that Cx26 
      is involved in the maturation of the cytoskeleton during the development of the 
      OC at the early postnatal stage. The polymerization of G-actin into F-actin is 
      prevented in Cx26 KD mice.
CI  - Copyright (c) 2022 Liu, Jin, Chen, Xu, Xie, Qiu, Wang, Sun and Kong.
FAU - Liu, Xiao-Zhou
AU  - Liu XZ
AD  - Department of Otorhinolaryngology, Union Hospital, Tongji Medical College, 
      Huazhong University of Science and Technology, Wuhan, China.
FAU - Jin, Yuan
AU  - Jin Y
AD  - Department of Otorhinolaryngology, Union Hospital, Tongji Medical College, 
      Huazhong University of Science and Technology, Wuhan, China.
FAU - Chen, Sen
AU  - Chen S
AD  - Department of Otorhinolaryngology, Union Hospital, Tongji Medical College, 
      Huazhong University of Science and Technology, Wuhan, China.
FAU - Xu, Kai
AU  - Xu K
AD  - Department of Otolaryngology, Head and Neck Surgery, The Second Affiliated 
      Hospital of Nanchang University, Nanchang, China.
FAU - Xie, Le
AU  - Xie L
AD  - Department of Otorhinolaryngology, Union Hospital, Tongji Medical College, 
      Huazhong University of Science and Technology, Wuhan, China.
FAU - Qiu, Yue
AU  - Qiu Y
AD  - Department of Otorhinolaryngology, Union Hospital, Tongji Medical College, 
      Huazhong University of Science and Technology, Wuhan, China.
FAU - Wang, Xiao-Hui
AU  - Wang XH
AD  - Department of Otorhinolaryngology, Union Hospital, Tongji Medical College, 
      Huazhong University of Science and Technology, Wuhan, China.
FAU - Sun, Yu
AU  - Sun Y
AD  - Department of Otorhinolaryngology, Union Hospital, Tongji Medical College, 
      Huazhong University of Science and Technology, Wuhan, China.
AD  - Institute of Otorhinolaryngology, Tongji Medical College, Huazhong University of 
      Science and Technology, Wuhan, China.
FAU - Kong, Wei-Jia
AU  - Kong WJ
AD  - Department of Otorhinolaryngology, Union Hospital, Tongji Medical College, 
      Huazhong University of Science and Technology, Wuhan, China.
AD  - Institute of Otorhinolaryngology, Tongji Medical College, Huazhong University of 
      Science and Technology, Wuhan, China.
LA  - eng
PT  - Journal Article
DEP - 20220307
PL  - Switzerland
TA  - Front Mol Neurosci
JT  - Frontiers in molecular neuroscience
JID - 101477914
PMC - PMC8957075
OTO - NOTNLM
OT  - GJB2
OT  - cochlear development
OT  - connexin26
OT  - f-actin
OT  - hearing loss
COIS- The authors declare that the research was conducted in the absence of any 
      commercial or financial relationships that could be construed as a potential 
      conflict of interest.
EDAT- 2022/03/30 06:00
MHDA- 2022/03/30 06:01
PMCR- 2021/01/01
CRDT- 2022/03/29 05:15
PHST- 2021/11/03 00:00 [received]
PHST- 2021/12/13 00:00 [accepted]
PHST- 2022/03/29 05:15 [entrez]
PHST- 2022/03/30 06:00 [pubmed]
PHST- 2022/03/30 06:01 [medline]
PHST- 2021/01/01 00:00 [pmc-release]
AID - 10.3389/fnmol.2021.808553 [doi]
PST - epublish
SO  - Front Mol Neurosci. 2022 Mar 7;14:808553. doi: 10.3389/fnmol.2021.808553. 
      eCollection 2021.