Vision Seminar Hosts an International Guest Speaker

On Thursday February 16, 2017 the Vision Seminar Series was very pleased to host its first international guest speaker, Shadi Safuri M.D., Ph.D., who is currently an ophthalmology resident at the at Rambam Medical Center in Haifa, Israel. Dr. Safuri came to UMass to complete an Advanced Ocular Imaging Rotation within the Department of Ophthalmology & Visual Sciences at the Eye Center at UMass Memorial Healthcare.

Dr. Safuri presented his research work performed at the Technion - Israel Institute of Technology Faculty of Medicine, under the mentorship of Professor Perlman. This work focused on better understanding of the pathogenesis of macular degeneration in an in-vitro model of Best Disease. Dr. Safuri also discussed factors underlying macular susceptibility.

Macular degeneration is a group of diseases characterized by loss of central vision and decline of visual acuity while visual field remains intact. Best-Disease is an inherited form, caused by mutation in bestrophin-1 gene. Interestingly manifestation of the lesion is restricted to the macula despite the fact that mutated bestrophin is present in the entire retina.

Dr. Safuri’s innovative work demonstrated that bestrophin-1 mutations modulate phagocytosis of shed photoreceptors outer segments in a phenotype dependent manner, proposing that wild-type bestrophin-1 tune down phagocytosis rate to prevent transient accumulation of high levels of toxic precursors that favor lipofuscin accumulation.

Moreover, his work demonstrated that retinal pigment epithelium cells facing cone photoreceptors display distinct gene expression profile compared to those facing rod photoreceptors, including genes coding for proteins of the complement system. Given that the macula has higher density of cone photoreceptors, this finding can explain, in part, macular susceptibility to age related lesions and other forms of macular degeneration.