Linked Life Data

16481432

Source:http://linkedlifedata.com/resource/pubmed/id/16481432

Statements in which the resource exists as a subject.
PredicateObject
rdf:type
lifeskim:mentions
pubmed:issue
7
pubmed:dateCreated
2006-2-16
pubmed:abstractText
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.
pubmed:grant
pubmed:language
eng
pubmed:journal
pubmed:citationSubset
IM
pubmed:chemical
pubmed:status
MEDLINE
pubmed:month
Feb
pubmed:issn
1529-2401
pubmed:author
pubmed:issnType
Electronic
pubmed:day
15
pubmed:volume
26
pubmed:owner
NLM
pubmed:authorsComplete
Y
pubmed:pagination
1991-9
pubmed:dateRevised
2007-11-14
pubmed:meshHeading
pubmed:year
2006
pubmed:articleTitle
Connexin29 is highly expressed in cochlear Schwann cells, and it is required for the normal development and function of the auditory nerve of mice.
pubmed:affiliation
Department of Otolaryngology, Emory University School of Medicine, Atlanta, Georgia 30322, USA.
pubmed:publicationType
Journal Article, Research Support, Non-U.S. Gov't, Research Support, N.I.H., Extramural