Medical Student Research Fellowship for Summer 2005

Mentor: Wojciech Kedzierski, Ph.D., D.Sc.
Department: Ophthalmology
Room number: E7.239
Mail Code: 9057
Phone number: 214-648-4556
E-mail: Wojciech Kedzierski
Project title: Does pathogenic Elovl4 mutation induce photoreceptor ER stress in mouse model of dominant Stargardt-like macular dystrophy?

Human subjects IRB approved project number (where applicable): not applicable

Animal subjects IRB approved project number (where applicable): APN # 0970-02-02

Project Type (patient-based research, animal-based research, or basic research; this characterization is only to permit a general classification for grouping similar types of projects):
animal-based research

Brief Description of Project:
Five-bp deletion in the coding end of human ELOVL4 gene is a cause of dominant Stargardt-like macular dystrophy. We are generating Stgd3-mice, a model of the disease, in order to determine its pathogenic pathways. One of several possible pathogenic mechanisms may involve cellular stress caused by the mutated Elovl4 protein. Such aberrant protein can form misfolded molecules and protein aggregates that are degraded in photoreceptors by unfolded protein pathway. To test this hypothesis, we propose to collect mouse retinas from wild-type and Stgd3-mice and to determine the levels of proteins that are cellular components of the unfolded protein response. In particular, using Western blot analysis we will study ATF6 and BiP that are up-regulated in cells by unfolded proteins. After we learn more about the pathogenic mechanisms in the Stgd3-mice, we will try to design treatments that block these pathogenic pathways in our Stgd3-mice.

Previous Research Activities or Publications with Medical Students:
Right now I help one 2-nd year medical student and one 4-year medical student in their research projects carried on at the Department of Ophthalmology.

Return to UT Southwestern Homepage

Return to Student Research Projects Index