Hair cell bundles, with and without a functional stereocilin protein (IMAGE)
Caption
This scanning electron micrograph shows sensory outer hair cells, which are required for cochlear amplification and normal hearing. The image at left shows a disorganized sensory hair cell from the inner ear from a mouse with a mutation in the Strc gene; as a result, the cell is missing scaffolding links provided by the stereocilin protein. A neighboring hair cell, at right, took up the dual-AAV gene therapy vector and has its normal organization restored. The red arrows point out the restored stereocilin cross-links, which stand the hair cell microvilli up into organized bundles that can contact the overlying tectorial membrane and detect sound vibrations.
Credit
Carl Nist-Lund, Holt/Géléoc Lab, Boston Children’s Hospital
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