The photoreceptor outer segment is an elaborate primary cilium. The delivery of opsin into and along the cilium is critical for photoreceptor cell function and viability. The overall goal of this study is to understand the cellular mechanisms involved in these delivery processes. A combination of cell biological methods, including imaging of protein movements in the cilium of live mouse photoreceptor cells, will be used. Studies will address questions about opsin entry into and transport along the cilium, and the membrane diffusion barrier of the photoreceptor cilium. The proposed research incorporates technical innovation, including the measurements of real-time movements of opsin along the cilium, using a ciliated epithelial cell line and mouse rod photoreceptor cells. It will test novel concepts of opsin trafficking and the organization of the photoreceptor cilium.

Public Health Relevance

The proposed research will provide a fundamental mechanistic understanding of cellular processes in the photoreceptor cilium. It will provide insight into the pathogenesis of a wide range of diseases, known as ciliopathies, which include retinal degeneration. There will also be tangible benefits to preclinical studies for retinal degeneration therapies, in that mutant phenotypes, which can be used to assess therapeutic efficacy, are likely to be identified by the research.

Agency
National Institute of Health (NIH)
Institute
National Eye Institute (NEI)
Type
Research Project (R01)
Project #
5R01EY013408-08
Application #
8656112
Study Section
(BVS)
Program Officer
Neuhold, Lisa
Project Start
2001-09-30
Project End
2018-04-30
Budget Start
2014-05-01
Budget End
2015-04-30
Support Year
8
Fiscal Year
2014
Total Cost
Indirect Cost
Name
University of California Los Angeles
Department
Ophthalmology
Type
Schools of Medicine
DUNS #
City
Los Angeles
State
CA
Country
United States
Zip Code
90095
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