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View Annual Report - Jules Stein Eye Institute

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30 Faculty | Bok<br />

Dean Bok, PhD<br />

Dolly Green Professor of Ophthalmology<br />

Distinguished Professor of Neurobiology<br />

Member of the <strong>Jules</strong> <strong>Stein</strong> <strong>Eye</strong> <strong>Institute</strong><br />

Member of the Brain Research <strong>Institute</strong><br />

ReseaRch summaRy<br />

Cell and Molecular Biology<br />

of the Retina<br />

Dr. Bok’s research interests involve the cell and molecular<br />

biology of the normal and diseased retina. In one<br />

research area, he is identifying and characterizing<br />

genes specific to retinal pigment epithelium (RPE) and<br />

exploring interactions that take place between RPE and<br />

retinal photoreceptors. The RPE performs a multitude<br />

of functions in the retina, including the transport of<br />

nutrients, ions, and fluid; the uptake and processing of<br />

vitamin A; and the daily removal of outer segment disc<br />

membranes that have been discarded by the photoreceptors.<br />

A second area of research involves the study<br />

of animal models of human retinitis pigmentosa and<br />

macular degeneration.<br />

Dr. Bok is using the techniques of cell and molecular<br />

biology to determine the proteins responsible for<br />

photoreceptor degeneration. One of the proteins under<br />

study in mice and humans is rds/peripherin. Because of<br />

a gene mutation, this protein is defective in a strain of<br />

mice called rds. As a result, the photoreceptors fail to<br />

form their light-sensitive organelles and eventually die.<br />

Dr. Bok and his collaborators have prevented blindness<br />

in these mice by injecting an artificial gene for rds/<br />

peripherin that performs normally. They are currently<br />

placing human rds/peripherin mutations into mice in<br />

order to study the mechanisms that cause photoreceptor<br />

death. Attempts are being made to slow the<br />

process of photoreceptor degeneration by delivery of<br />

neurotrophic factors into the retina by nonpathogenic<br />

viruses. Finally, with new information regarding the<br />

genetics of age-related macular degeneration, Dr. Bok<br />

and collaborators are studying mechanisms whereby<br />

the alternative complement pathway of the immune<br />

system contributes to this disease.<br />

Public Service<br />

Scientific Advisory Board Member, E. Matilda Ziegler<br />

Foundation for the Blind, the Karl Kirchgessner Foundation,<br />

the Foundation Fighting Blindness, and the Macula Vision<br />

Research Foundation<br />

External Advisory Board Member, Center of Biomedical<br />

Research Excellence, University of Oklahoma<br />

Health Sciences Center; and the Macular Telangiectasia<br />

Project, Lowy Medical Research <strong>Institute</strong>, LTD<br />

Editorial Board Member, International Review of Cytology<br />

Reviewer for many scientific journals<br />

Research Grants<br />

Macula Vision Research Foundation: Identification and<br />

Cellular Localization of Gene Products that Affect<br />

Photoreceptor Survival in Inherited Retinal Degeneration,<br />

4/1/08–3/31/12<br />

National <strong>Eye</strong> <strong>Institute</strong>: Development of Complement<br />

Modulating Therapeutics for AMD (Principal Investigator:<br />

Gregory S. Hageman, PhD, with other investigators),<br />

8/1/06–7/31/11<br />

National <strong>Eye</strong> <strong>Institute</strong>: RDS Mutations; Gene Therapy for<br />

ADRP, Macular Degeneration, and Pattern Dystrophy<br />

(Principal Investigator: Alfred S. Lewin, PhD, with other<br />

investigators), 9/1/07–8/31/11<br />

California <strong>Institute</strong> for Regenerative Medicine: Development<br />

of a Stem Cell-Based Transplantation Strategy for Treating<br />

Age-Related Macular Degeneration (Principal Investigator:<br />

Gabriel Travis, MD, with other investigators),<br />

11/1/09–10/31/12

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