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
Related in: MedlinePlus
Mentions: To examine in greater detail stereocilia and hair cell morphology in Pls1 KO mice, we used scanning (SEM) or transmission (TEM) electron microscopy. In young (P12) animals, the overall organization of the organ of Corti was unchanged in Pls1 KO mice compared with wt littermates (Fig. 3A and B). The stereociliary bundles of hair cells displayed a characteristic straight (for IHC) or V-shape (for OHC) and were of uniform orientation. The cuticular plates of plastin 1-deficient IHCs and OHCs developed normally and showed similar thickness, spectrin expression and actin content to those of wt hair cells (Fig. 4C–N).Figure 4.
Affiliation: Centre for Auditory Research, UCL Ear Institute, University College London, London, UK.