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The Journal of Neuroscience, August 1, 1999, 19(15):6267-6274
Myosin VIIa Participates in Opsin Transport through The
Photoreceptor Cilium
Xinran
Liu1,
Igor P.
Udovichenko1,
Stephen D.M.
Brown2,
Karen P.
Steel3, and
David S.
Williams1
1 Departments of Pharmacology and Neurosciences,
University of California at San Diego School of Medicine, La
Jolla, California 92093-0983, 2 Medical Research Council
Mammalian Genetics Unit and Mouse Genome Centre, Harwell OX11 0RD,
United Kingdom, and 3 Medical Research Council Institute of
Hearing Research, University Park, Nottingham NG7 2RD, United Kingdom
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 the
connecting cilium of each photoreceptor cell and participates in the
transport of opsin through this structure. These findings provide the
first direct evidence that opsin travels along the connecting cilium en
route to the outer segment. They demonstrate that a myosin may function
in a cilium and suggest that abnormal opsin transport might contribute
to blindness in Usher syndrome.
Key words:
myosin; molecular motor; cilium; photoreceptor; opsin; Usher syndrome
Copyright © 1999 Society for Neuroscience 0270-6474/99/19156267-08$05.00/0
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|
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|
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[Full Text]
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|
 |
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|
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|
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|
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[Full Text]
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|
 |
|

|
 |

|
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[Full Text]
[PDF]
|
 |
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|
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|
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[Full Text]
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|
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[Full Text]
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[Full Text]
[PDF]
|
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|

|
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|
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97(7):
3649 - 3654.
[Abstract]
[Full Text]
[PDF]
|
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|
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[Full Text]
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|
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