PMID- 16481432
OWN - NLM
STAT- MEDLINE
DCOM- 20060424
LR  - 20200225
IS  - 1529-2401 (Electronic)
IS  - 0270-6474 (Print)
IS  - 0270-6474 (Linking)
VI  - 26
IP  - 7
DP  - 2006 Feb 15
TI  - Connexin29 is highly expressed in cochlear Schwann cells, and it is required for 
      the normal development and function of the auditory nerve of mice.
PG  - 1991-9
AB  - Connexins (Cxs) are a family of protein subunits constituting gap junctions, 
      which facilitate exchanges of molecules important for cellular signaling and 
      metabolic activities intercellularly or between different regions of the 
      cytoplasm in the same cells. Mutations in Cxs are the major cause of nonsyndromic 
      childhood deafness, which are mostly found in Cx26 and Cx30 expressed in cochlear 
      supporting cells and fibrocytes. So far, little is known about the functional 
      contribution of Cxs in other types of cochlear cells. Here, we show that Cx29 was 
      highly expressed in the cochlea. The developmental expression time course of Cx29 
      was similar to that of a myelin marker [myelin associate glycoprotein (MAG)]. 
      Immunolabeling identified Cx29 exclusively in the Schwann cells myelinating the 
      soma and fiber of spiral ganglion (SG) neurons. The absence of the Cx29 gene in 
      mice (Cx29(-/-) mice), with a penetrance of approximately 50%, caused a delay in 
      the maturation of hearing thresholds, an early loss of high-frequency 
      sensitivities, a prolongation in latency and distortion in the wave I of the 
      auditory brainstem responses, and elevated sensitivity to noise damages. The 
      morphology of sensory hair cells and otoacoustic emissions that depend on the 
      integrity of hair cells were normal in Cx29(-/-) mice. In contrast, decreases in 
      MAG expression and severe demyelination at the soma of SG neurons were found in 
      Cx29(-/-) mice. Our findings demonstrated the requirement of Cx29 for normal 
      cochlear functions and suggest that Cx29 is a new candidate gene for studying the 
      auditory neuropathy.
FAU - Tang, Wenxue
AU  - Tang W
AD  - Department of Otolaryngology, Emory University School of Medicine, Atlanta, 
      Georgia 30322, USA.
FAU - Zhang, Yanping
AU  - Zhang Y
FAU - Chang, Qing
AU  - Chang Q
FAU - Ahmad, Shoab
AU  - Ahmad S
FAU - Dahlke, Ian
AU  - Dahlke I
FAU - Yi, Hong
AU  - Yi H
FAU - Chen, Ping
AU  - Chen P
FAU - Paul, David L
AU  - Paul DL
FAU - Lin, Xi
AU  - Lin X
LA  - eng
GR  - R01-DC04709/DC/NIDCD NIH HHS/United States
GR  - GM37751/GM/NIGMS NIH HHS/United States
GR  - R01-DC006483/DC/NIDCD NIH HHS/United States
GR  - R01 DC006483/DC/NIDCD NIH HHS/United States
GR  - P30-HD18655/HD/NICHD NIH HHS/United States
PT  - Journal Article
PT  - Research Support, N.I.H., Extramural
PT  - Research Support, Non-U.S. Gov't
PL  - United States
TA  - J Neurosci
JT  - The Journal of neuroscience : the official journal of the Society for 
      Neuroscience
JID - 8102140
RN  - 0 (Connexins)
RN  - 0 (Gje1 protein, mouse)
RN  - 0 (Nerve Tissue Proteins)
SB  - IM
MH  - Animals
MH  - Cochlea/*physiology
MH  - Cochlear Nerve/*physiology
MH  - Connexins/deficiency/*genetics
MH  - Gap Junctions/physiology
MH  - Mice
MH  - Mice, Knockout
MH  - Myelin Sheath/physiology
MH  - Nerve Tissue Proteins/deficiency/*genetics
MH  - Schwann Cells/*physiology
PMC - PMC6674919
EDAT- 2006/02/17 09:00
MHDA- 2006/04/25 09:00
PMCR- 2006/08/15
CRDT- 2006/02/17 09:00
PHST- 2006/02/17 09:00 [pubmed]
PHST- 2006/04/25 09:00 [medline]
PHST- 2006/02/17 09:00 [entrez]
PHST- 2006/08/15 00:00 [pmc-release]
AID - 26/7/1991 [pii]
AID - 10.1523/JNEUROSCI.5055-05.2006 [doi]
PST - ppublish
SO  - J Neurosci. 2006 Feb 15;26(7):1991-9. doi: 10.1523/JNEUROSCI.5055-05.2006.