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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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Related in: MedlinePlus

Hair cell loss is not responsible for the hearing deficit in Pls1 KO mice. (A) Whole-mount, surface views of the organ of Corti of 6-week-old wt and Pls1 KO mice immunostained for parvalbumin. The vast majority of hair cell bodies are present in the medial and basal turn of the cochlea. Arrowhead points to the site of one missing OHC in the Pls1 KO sample. (B) Surface views of the organ of Corti of 4-month-old wt and Pls1 KO mice immunostained for myosin-VIIa . The IHC are well preserved in both the Pls1 KO and wt mice, but some OHCs are missing in wt and Pls1 KO mice (arrowheads), with increased frequency of OHC losses in the basal turns of the cochlea.
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DDU417F3: Hair cell loss is not responsible for the hearing deficit in Pls1 KO mice. (A) Whole-mount, surface views of the organ of Corti of 6-week-old wt and Pls1 KO mice immunostained for parvalbumin. The vast majority of hair cell bodies are present in the medial and basal turn of the cochlea. Arrowhead points to the site of one missing OHC in the Pls1 KO sample. (B) Surface views of the organ of Corti of 4-month-old wt and Pls1 KO mice immunostained for myosin-VIIa . The IHC are well preserved in both the Pls1 KO and wt mice, but some OHCs are missing in wt and Pls1 KO mice (arrowheads), with increased frequency of OHC losses in the basal turns of the cochlea.

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.


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)

Hair cell loss is not responsible for the hearing deficit in Pls1 KO mice. (A) Whole-mount, surface views of the organ of Corti of 6-week-old wt and Pls1 KO mice immunostained for parvalbumin. The vast majority of hair cell bodies are present in the medial and basal turn of the cochlea. Arrowhead points to the site of one missing OHC in the Pls1 KO sample. (B) Surface views of the organ of Corti of 4-month-old wt and Pls1 KO mice immunostained for myosin-VIIa . The IHC are well preserved in both the Pls1 KO and wt mice, but some OHCs are missing in wt and Pls1 KO mice (arrowheads), with increased frequency of OHC losses in the basal turns of the cochlea.
© Copyright Policy - creative-commons
Related In: Results  -  Collection

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Show All Figures
getmorefigures.php?uid=PMC4262491&req=5

DDU417F3: Hair cell loss is not responsible for the hearing deficit in Pls1 KO mice. (A) Whole-mount, surface views of the organ of Corti of 6-week-old wt and Pls1 KO mice immunostained for parvalbumin. The vast majority of hair cell bodies are present in the medial and basal turn of the cochlea. Arrowhead points to the site of one missing OHC in the Pls1 KO sample. (B) Surface views of the organ of Corti of 4-month-old wt and Pls1 KO mice immunostained for myosin-VIIa . The IHC are well preserved in both the Pls1 KO and wt mice, but some OHCs are missing in wt and Pls1 KO mice (arrowheads), with increased frequency of OHC losses in the basal turns of the cochlea.
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.

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