Role of myosin VI in the differentiation of cochlear hair cells

Tim Self, Tama Sobe, Neal G. Copeland, Nancy A. Jenkins, Karen B. Avraham, Karen P. Steel*

*Corresponding author for this work

Research output: Contribution to journalArticlepeer-review


The mouse mutant Snell's waltzer (sv) has an intragenic deletion of the Myo6 gene, which encodes the unconventional myosin molecule myosin VI (K. B. Avraham et al., 1995, Nat. Genet. 11, 369-375). Snell's waltzer mutants exhibit behavioural abnormalities suggestive of an inner ear defect, including lack of responsiveness to sound, hyperactivity, head tossing, and circling. We have investigated the effects of a lack of myosin VI on the development of the sensory hair cells of the cochlea in these mutants. In normal mice, the hair cells sprout microvilli on their upper surface, and some of these grow to form a crescent or V-shaped array of modified microvilli, the stereocilia. In the mutants, early stages of stereocilia development appear to proceed normally because at birth many stereocilia bundles have a normal appearance, but in places there are signs of disorganisation of the bundles. Over the next few days, the stereocilia become progressively more disorganised and fuse together. Practically all hair cells show fused stereocilia by 3 days after birth, and there is extensive stereocilia fusion by 7 days. By 20 days, giant stereocilia are observed on top of the hair cells. At 1 and 3 days after birth, hair cells of mutants and controls take up the membrane dye FM1-43, suggesting that endocytosis occurs in mutant hair cells. One possible model for the fusion is that myosin VI may be involved in anchoring the apical hair cell membrane to the underlying actin-rich cuticular plate, and in the absence of normal myosin VI this apical membrane will tend to pull up between stereocilia, leading to fusion.

Original languageEnglish
Pages (from-to)331-341
Number of pages11
JournalDevelopmental Biology
Issue number2
StatePublished - 15 Oct 1999


FundersFunder number
Defeating Deafness
Israel Academy of Arts and Sciences
Medical Research Council
British Skin Foundation
European CommissionCT97-2715
United States-Israel Binational Science Foundation
Israel Science Foundation


    • Cochlear hair cells
    • Genetic deafness
    • Microvilli
    • Mouse mutant
    • Myosin VI
    • Snell's waltzer
    • Unconventional myosin


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