Molecular Mechanisms in Inner Ear Development

In vitro differentiated hair cells. Featured on the cover of Developmental Biology (2010).

Cells are built from proteins, and specialized cells such as hair cells express a unique complement of proteins. Consider that cochlear sensory cells in humans survive for decades; consider too that hearing is an active process, where sound waves are amplified or suppressed through the function of the outer hair cells, also known as the cochlear amplifier. Is it any wonder that over a hundred genetic loci for hearing loss have been found? Identifying what genes are necessary for hair cell or supporting cell survival forms the basis for understanding how these cells develop and function.

Using cell sorting techniques, we have previously obtained purified populations of hair cells, supporting cells and progenitor cells; we then analyzed the transcriptomes of these populations with Affymetrix arrays. From this data I have generated lists of genes that are likely expressed in each cell type. Any of these genes may be analyzed through expression analysis, loss of function and gain of function techniques.

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