Connexin29 Is Highly Expressed in Cochlear Schwann Cells, and It Is Required for the Normal Development and Function of the Auditory Nerve of Mice

• DOI: 10.1523/jneurosci.5055-05.2006
• Publication Date: 2006-02-15T11:52:49Z
• Authors: Wenxue Tang; Yanping Zhang; Qing Chang; Shoab Ahmad; Ian Dahlke; Hong Yi; Ping Chen; David L. Paul; Xi Lin
• Keywords: Spiral ganglion; Immunolabeling; Auditory neuropathy; Peripherin; Cochlear nerve

Connexins (Cxs) are a family of protein subunits constituting gap junctions, which facilitate exchanges molecules important for cellular signaling and metabolic activities intercellularly or between different regions the cytoplasm in same cells. Mutations Cxs major cause nonsyndromic childhood deafness, mostly found Cx26 Cx30 expressed cochlear supporting cells fibrocytes. So far, little is known about functional contribution other types Here, we show that Cx29 was highly cochlea. The developmental expression time course similar to myelin marker [myelin associate glycoprotein (MAG)]. Immunolabeling identified exclusively Schwann myelinating soma fiber spiral ganglion (SG) neurons. absence gene mice (Cx29 −/− mice), with penetrance ∼50%, caused delay maturation hearing thresholds, an early loss high-frequency sensitivities, prolongation latency distortion wave I auditory brainstem responses, elevated sensitivity noise damages. morphology sensory hair otoacoustic emissions depend on integrity were normal mice. In contrast, decreases MAG severe demyelination at SG neurons Our findings demonstrated requirement functions suggest new candidate studying neuropathy.