Dean Bok, PhD, Director
Phone: (310) 825-6737
Fax: (310) 794-2144
Email: [email protected]
100 Stein Plaza, Room B-182
Los Angeles, CA 90095
Cell Biology of the Retina
Molecular Biology of the Retina
Dr. Bok's research interests involve the cell and molecular biology of the normal and diseased retina. In one research area, he is identifying and characterizing genes specific to retinal pigment epithelium (RPE) and exploring interactions that take place between RPE and retinal photoreceptors. The RPE performs a multitude of functions in the retina, including the transport of nutrients, ions and fluid; the uptake and processing of vitamin A; and the daily removal of outer segment disc membranes that have been discarded by the photoreceptors. A second research area involves the study of animal models for human retinitis pigmentosa and macular degeneration. Dr. Bok is using the techniques of cell and molecular biology to determine the proteins responsible for photoreceptor degeneration. One of the proteins under study in mice and humans is rds/peripherin. Because of a gene mutation, this protein is defective in a strain of mice called rds. As a result, the photoreceptors fail to form their light-sensitive organelles and eventually die. Dr. Bok and collaborators have prevented blindness in these mice by injecting an artificial gene for rds/peripherin that performs normally. They are currently placing human rds/peripherin mutations into mice in order to study the mechanisms that cause photoreceptor death. Attempts are being made to slow the process of photoreceptor degeneration by delivery of neurotrophic factors into the retina by nonpathogenic viruses.
Bok D, Ruiz A, Yaron O, Jahng WJ, Ray A, Xue LL, Rando R. Purification and characterization of a transmembrane domain-deleted form of lecithin retinol acyltransferase. Biochem 2003;42:6090-8.
Kedzierski W, Bok D, Travis GH. Non cell-autonomous photoreceptor degeneration in rds mutant mice mosaic for expression of a rescue transgene. J Neurosci 1998;8:4076-82.
Redmond TM, Yu S, Lee E, Bok D, Hamasaki D, Chen N, Goletz P, Ma J-X, Crouch RK, Pfeifer K. RPE65 is necessary for production of 11-cis-Vitamin A in the retinal visual cycle. Nat Genet 1998;20:344-51.
Ruiz A, Winston A, Lim Y-H, Gilbert BA, Rando R, Bok D. Molecular and biochemical characterization of lecithin retinol acyltransferase. J Biol Chem 1999;274:3834-41.
Bok D, Galbraith G, Lopez I, Woodruff M, Nusinowitz S, Beltrand delRio H, Juang W, Zhao S, Geski R, Montgomery C, Van Slightenhorst E, Friddle C, Platt K, Sparks MJ, Pushkin A, Abuladze N, Ishiyama A, Dukkipati R, Liu W and Kurtz I. Blindness and deafness caused by loss of sodium bicarbonate cotransport. Nature Genetics 2003;34:313-9.
The Foundation Fighting Blindness National Retinitis Pigmentosa Foundation Center Grant (Center Coordinator): 7/1/00-6/30/05 (with other Investigators)
National Eye Institute: The Pathology of Inherited Retinal Disease, 12/1/01-11/30/06