Extracellular matrix dysfunction in Sorsby patient-derived retinal pigment epithelium
AL Engel, YK Wang, TH Khuu, E Worrall… - Experimental eye …, 2022 - Elsevier
Abstract Sorsby Fundus Dystrophy (SFD) is a rare form of macular degeneration that is
clinically similar to age-related macular degeneration (AMD), and a histologic hallmark of
SFD is a thick layer of extracellular deposits beneath the retinal pigment epithelium (RPE).
Previous studies of SFD patient-induced pluripotent stem cell (iPSC) derived RPE differ as to
whether these cultures recapitulate this key clinical feature by forming increased drusenoid
deposits. The primary purpose of this study is to examine whether SFD patient-derived iPSC …
clinically similar to age-related macular degeneration (AMD), and a histologic hallmark of
SFD is a thick layer of extracellular deposits beneath the retinal pigment epithelium (RPE).
Previous studies of SFD patient-induced pluripotent stem cell (iPSC) derived RPE differ as to
whether these cultures recapitulate this key clinical feature by forming increased drusenoid
deposits. The primary purpose of this study is to examine whether SFD patient-derived iPSC …
