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Absence of plastin 1 causes abnormal maintenance of hair cell stereocilia and a moderate form of hearing loss in mice.

Taylor R, Bullen A, Johnson SL, Grimm-Günter EM, Rivero F, Marcotti W, Forge A, Daudet N - Hum. Mol. Genet. (2014)

Bottom Line: Several actin-associated proteins are essential for stereocilia formation and maintenance, and their absence leads to deafness.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.

View Article: PubMed Central - PubMed

Affiliation: Centre for Auditory Research, UCL Ear Institute, University College London, London, UK.

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Auditory hair cells develop normally in young Pls1 KO mice. (A and B) Scanning electron micrographs of the surface of the organ of Corti in the medial turn of the cochlea of P12 wt (A) and Pls1 KO (B) mice. (C–F) Transmission electron micrographs of the apical region of the IHC and OHC in P12 wt and Pls1 KO mice. The contour of the cuticular plate (CP) is outlined by a white dotted line. (G–J) Surface views of the IHCs and OHCs of P12 wt and Pls1 KO mice immunostained for spectrin, a marker of the CP. (K–N) Surface views of the IHCs and OHCs of P30 wt and Pls1 KO mice stained with fluorescent phalloidin. The F-actin content of stereocilia and the CP appears similar in wt and Pls1 KO hair cells.
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DDU417F4: Auditory hair cells develop normally in young Pls1 KO mice. (A and B) Scanning electron micrographs of the surface of the organ of Corti in the medial turn of the cochlea of P12 wt (A) and Pls1 KO (B) mice. (C–F) Transmission electron micrographs of the apical region of the IHC and OHC in P12 wt and Pls1 KO mice. The contour of the cuticular plate (CP) is outlined by a white dotted line. (G–J) Surface views of the IHCs and OHCs of P12 wt and Pls1 KO mice immunostained for spectrin, a marker of the CP. (K–N) Surface views of the IHCs and OHCs of P30 wt and Pls1 KO mice stained with fluorescent phalloidin. The F-actin content of stereocilia and the CP appears similar in wt and Pls1 KO hair cells.

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.


Absence of plastin 1 causes abnormal maintenance of hair cell stereocilia and a moderate form of hearing loss in mice.

Taylor R, Bullen A, Johnson SL, Grimm-Günter EM, Rivero F, Marcotti W, Forge A, Daudet N - Hum. Mol. Genet. (2014)

Auditory hair cells develop normally in young Pls1 KO mice. (A and B) Scanning electron micrographs of the surface of the organ of Corti in the medial turn of the cochlea of P12 wt (A) and Pls1 KO (B) mice. (C–F) Transmission electron micrographs of the apical region of the IHC and OHC in P12 wt and Pls1 KO mice. The contour of the cuticular plate (CP) is outlined by a white dotted line. (G–J) Surface views of the IHCs and OHCs of P12 wt and Pls1 KO mice immunostained for spectrin, a marker of the CP. (K–N) Surface views of the IHCs and OHCs of P30 wt and Pls1 KO mice stained with fluorescent phalloidin. The F-actin content of stereocilia and the CP appears similar in wt and Pls1 KO hair cells.
© Copyright Policy - creative-commons
Related In: Results  -  Collection

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getmorefigures.php?uid=PMC4262491&req=5

DDU417F4: Auditory hair cells develop normally in young Pls1 KO mice. (A and B) Scanning electron micrographs of the surface of the organ of Corti in the medial turn of the cochlea of P12 wt (A) and Pls1 KO (B) mice. (C–F) Transmission electron micrographs of the apical region of the IHC and OHC in P12 wt and Pls1 KO mice. The contour of the cuticular plate (CP) is outlined by a white dotted line. (G–J) Surface views of the IHCs and OHCs of P12 wt and Pls1 KO mice immunostained for spectrin, a marker of the CP. (K–N) Surface views of the IHCs and OHCs of P30 wt and Pls1 KO mice stained with fluorescent phalloidin. The F-actin content of stereocilia and the CP appears similar in wt and Pls1 KO hair cells.
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.

Bottom Line: Several actin-associated proteins are essential for stereocilia formation and maintenance, and their absence leads to deafness.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.

View Article: PubMed Central - PubMed

Affiliation: Centre for Auditory Research, UCL Ear Institute, University College London, London, UK.

Show MeSH
Related in: MedlinePlus