Abstract by Theresa Smith
Chemistry and Biochemistry
The role of CCT in folding the crucial visual cycle protein, RPE65
RPE65 is a critical isomerase involved in the regeneration of 11-cis-retinal, a photosensitive molecule required for vision. Mutations in RPE65 are linked to mild to severe retinal degenerative diseases including Leber congenital amaurosis (LCA), which often leads to blindness in early childhood. However, little is known about how RPE65 gets folded. We are investigating a novel interaction between RPE65 and the cytosolic chaperonin, CCT. SgRNA knockdown of CCT results in reduced RPE65 expression indicating that it contributes to RPE65 folding. A high-resolution cryo-EM structure of this complex will help to fully elucidate the role of CCT and may contribute to an improved understanding of RPE65 pathogenesis.