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Identification of the Hair Cell Soma-1 Antigen, HCS-1, as Otoferlin

journal contribution
posted on 2023-06-07, 21:33 authored by Richard GoodyearRichard Goodyear, P Kevin Legan, Jeffrey R Christiansen, Bei Xia, Julia Korchagina, Jonathan E Gale, Mark E Warchol, Jeffrey T Corwin, Guy Richardson
Hair cells, the mechanosensitive receptor cells of the inner ear, are critical for our senses of hearing and balance. The small number of these receptor cells in the inner ear has impeded the identification and characterization of proteins important for hair cell function. The binding specificity of monoclonal antibodies provides a means for identifying hair cell-specific proteins and isolating them for further study. We have generated a monoclonal antibody, termed hair cell soma-1 (HCS-1), which specifically immunolabels hair cells in at least five vertebrate classes, including sharks and rays, bony fish, amphibians, birds, and mammals. We used HCS-1 to immunoprecipitate the cognate antigen and identified it as otoferlin, a member of the ferlin protein family. Mutations in otoferlin underlie DFNB9, a recessive, nonsyndromic form of prelingual deafness characterized as an auditory neuropathy. Using immunocytochemistry, we find that otoferlin is associated with the entire basolateral membrane of the hair cells and with vesicular structures distributed throughout most of the hair cell cytoplasm. Biochemical assays indicate that otoferlin is tightly associated with membranes, as it is not solubilized by alterations in calcium or salt concentrations. HCS-1 immunolabeling does not co-localize with ribeye, a constituent of synaptic ribbons, suggesting that otoferlin may, in addition to its proposed function in synaptic vesicle release, play additional roles in hair cells.

History

Publication status

  • Published

Journal

Journal of the Association for Research in Otolaryngology

ISSN

1525-3961

Publisher

Springer Verlag

Issue

4

Volume

11

Page range

573-586

Pages

14.0

Department affiliated with

  • Neuroscience Publications

Full text available

  • No

Peer reviewed?

  • Yes

Legacy Posted Date

2012-02-06

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