About Us » FFB Funded Researchers
Tiansen Li, Ph.D.
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Tiansen Li, Ph.D. is Associate Professor of Ophthalmology (Neuroscience), Department of Ophthalmology, Harvard Medical School in the Berman-Gund Laboratory for the Study of Retinal Degenerations at the Massachusetts Eye and Ear Infirmary. A graduate of Beijing Medical College, Beijing, China, he did post-doctoral training in the Visual Sciences Center, University of Chicago. He joined the Berman-Gund Laboratory in 1992. He has become a Principal Investigator with grant support from the NIH and the Foundation Fighting Blindness. He is the author or co-author of more than fifty peer-reviewed publications. He has three major areas of research interest. One is to investigate the functions of genes now known to be involved in the development of photoreceptor degeneration, including retinitis pigmentosa, Usher syndrome, and a more severe form of photoreceptor degeneration known as Leber congenital amaurosis (LCA). Genes under study have included RPGR (retinitis pigmentosa GTPase regulator), RPGRIP (RPGR interacting protein) and AIPL1 (aryl hydrocarbon receptor interacting protein 1). He and his colleagues generated the first murine model of X-linked retinitis pigmentosa through knockout of the RPGR gene and the first murine model of Usher syndrome, type 2A through knockout of the Ush2A gene. A second area of research focuses on developing therapies for degenerating photoreceptors. He and his co-workers have demonstrated that a single abbreviated RPGR variant was sufficient to reconstitute RPGR function in vivo. They have also conducted successful replacement gene therapy in a murine model of LCA lacking RPGRIP. A third area explores the pathogenesis of age-related macular degeneration (AM.D.). A goal is to understand the role of an intact Bruch’s membrane, which underlies the pigment epithelium, in preventing the progression of AM.D. to choroidal neovascularization (CNV). Homeostasis of the elastic fiber layer is an integral part in the normal maintenance of Bruch’s membrane. He and his colleagues have hypothesized that a decline in the structure/function of this layer due to advancing age and/or genetic predisposition may be a contributing factor to CNV. They have further hypothesized that the lysyl oxidase (LOX) family of proteins has a role in elastic fiber homeostasis. They have shown that lysyl oxidase-like 1 (LOXL1) is specifically localized along the elastic fibers and that mice lacking LOXL1 exhibit a marked defect in elastic fiber homeostasis. They plan to use this model to investigate the pathogenesis of CNV and consider possible preventative and therapeutic strategies. |
