CASK function in the inner ear

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Calcium/calmodulin-dependent serine kinase(CASK) plays an important role in brain development, and in patients with CASK mutations sensorineural hearing loss was observed. However, there has been no comprehensive study of CASK in the inner ear. To better understand the physiological importance of CASK, we examined the expression pattern of CASK in chick and mouse inner ear. We observed the expression of CASK in the hair cell(HC), the spiral ganglion(SG) neurons, and in the stria vascularis(SV). Analysis of CASK-binding protein identified some nuclear proteins, and the molecules required for membrane trafficking events. The cochlea from CASK conditional KO mice shows a clear defect with a decrease in the number of SG neurons and the morphology is disturbed. The data suggests that CASK is required for the formation and/or function of HC, SG neurons, and SV.