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Research Interests

Role of Barhl1 in the maintenance of cochlear hair cells. The cochlea of the mammalian inner ear contains three rows of outer hair cells and a single row of inner hair cells. These hair cell receptors reside in the organ of Corti and function to transduce mechanical stimuli into electrical signals that mediate hearing. To date, the molecular mechanisms underlying the maintenance of these delicate sensory hair cells are unknown. In a recent collaborative study, we have demonstrated that targeted disruption of the Barhl1 homeobox gene in mice causes severe to profound hearing loss as a result of age-related progressive degeneration of both outer and inner hair cells in the organ of Corti, following two reciprocal longitudinal gradients. Our data thus indicate an essential role for Barhl1 in the long-term maintenance of cochlear hair cells, but not in the determination or differentiation of these cells. Furthermore, our results suggest that Barhl1 deficient mice can serve as a unique model for the study of age-related human deafness disorders.

Loss of Barhl1 function results in progressive degeneration of cochlear hair cells. Development 129:3523-3532 (2002)

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