Absence of plastin 1 causes abnormal maintenance of hair cell stereocilia and a moderate form of hearing loss in mice.
Bottom Line: Auditory hair cells developed normally in Pls1 KO, but in young adult animals, the stereocilia of inner hair cells were reduced in width and length.These results show that in contrast to other actin-bundling proteins such as espin, harmonin or Eps8, plastin 1 is dispensable for the initial formation of stereocilia.Hence, mutations in the human PLS1 gene may be associated with relatively mild and progressive forms of hearing loss.
Affiliation: Centre for Auditory Research, UCL Ear Institute, University College London, London, UK.Show MeSH
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Mentions: To determine whether the auditory deficit in Pls1 KO mice might be due to hair cell loss, we analysed whole-mount or sectioned preparations of the organ of Corti labelled with phalloidin and hair cell-specific antibodies. In 6-week-old animals (Fig. 3A), the vast majority of inner (IHCs) and outer (OHCs) hair cells were present at all levels of the cochlea regardless of genotype, indicating that the absence of plastin 1 did not impair hair cell formation or survival. In 4-month-old animals (Fig. 3B), most IHCs and OHCs were present although a higher degree of OHC loss was noticed, particularly in the basal turn of Pls1 KO mice. These data indicate that the hearing loss found at all frequencies in young adult Pls1 KO animals is not consecutive to hair cell death.Figure 3.
Affiliation: Centre for Auditory Research, UCL Ear Institute, University College London, London, UK.