Myosin VIIa as a common component of cilia and microvilli.
نویسندگان
چکیده
The distribution of myosin VIIa, which is defective or absent in Usher syndrome 1B, was studied in a variety of tissues by immunomicroscopy. The primary aim was to determine whether this putative actin-based mechanoenzyme is a common component of cilia. Previously, it has been proposed that defective ciliary function might be the basis of some forms of Usher syndrome. Myosin VIIa was detected in cilia from cochlear hair cells, olfactory neurons, kidney distal tubules, and lung bronchi. It was also found to cofractionate with the axonemal fraction of retinal photoreceptor cells. Immunolabeling appeared most concentrated in the periphery of the transition zone of the cilia. This general presence of a myosin in cilia is surprising, given that cilia are dominated by microtubules, and not actin filaments. In addition to cilia, myosin VIIa was also found in actin-rich microvilli of different types of cell. We conclude that myosin VIIa is a common component of cilia and microvilli.
منابع مشابه
Myosin VIIa, the product of the Usher 1B syndrome gene, is concentrated in the connecting cilia of photoreceptor cells.
Usher syndrome is the most common form of combined deafness and blindness. The gene that is defective in Usher syndrome 1B (USH1B) encodes for an unconventional myosin, myosin VIIa. To understand the cellular function of myosin VIIa and why defects in it lead to USH1B, it is essential to determine the precise cellular and subcellular localization of the protein. We investigated the distribution...
متن کاملMyosin VIIa participates in opsin transport through the photoreceptor cilium.
Two types of Usher syndrome, a blindness-deafness disorder, result from mutations in the myosin VIIa gene. As for most other unconventional myosins, little is known about the function or functions of myosin VIIa. Here, we studied the photoreceptor cells of mice with mutant myosin VIIa by electron immunomicroscopy and microscopic autoradiography. We found evidence that myosin VIIa functions in t...
متن کاملCharacterization of the Drosophila Ortholog of the Human Usher Syndrome Type 1G Protein Sans
BACKGROUND The Usher syndrome (USH) is the most frequent deaf-blindness hereditary disease in humans. Deafness is attributed to the disorganization of stereocilia in the inner ear. USH1, the most severe subtype, is associated with mutations in genes encoding myosin VIIa, harmonin, cadherin 23, protocadherin 15, and sans. Myosin VIIa, harmonin, cadherin 23, and protocadherin 15 physically intera...
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Mutations in the myosin VIIa gene cause Usher syndrome type IB (USH1B), characterized by deaf-blindness. A delay of opsin trafficking has been observed in the retinal photoreceptor cells of myosin VIIa-deficient mice. We identified spectrin βV, the mammalian β-heavy spectrin, as a myosin VIIa- and rhodopsin-interacting partner in photoreceptor cells. Spectrin βV displays a polarized distributio...
متن کاملShaky hearing
Functional sensory hair cells in the inner ear have specialised microvilli — stereocilia — which are essential for hearing. The scanning electron micrograph at top shows the normal arrangement of stereocilia bundles on three outer hair cells in the inner ear of a three-day-old mouse. The stereocilia grow to form rows of graded height in a distinctive V-shaped bundle. Recent studies in mice have...
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ورودعنوان ژورنال:
- Cell motility and the cytoskeleton
دوره 40 3 شماره
صفحات -
تاریخ انتشار 1998