Retinoid isomerohydrolase

spinqualitylabelsall models Bovine RPE65 complex with palmitate, amixustat and Fe+2 ion (PDB code 4rsc) Show:Asymmetric Unit Biological Assembly Drag the structure with the mouse to rotate   Export Animated Image   Print3D Function Retinoid isomerohydrolase (RPE65) catalyzes the isomerization of trans-retinyl ester to 11-cis-retinol in the retinal pigment epithelium. This isomerization is a critical part of the visual cycle and is essential for normal vision. The RPE65 inhibitor amixustat is used as a drug which modulates visual cycle activity in age-related macular degeneration. RPE65 is iron-dependent. Disease Mutations in RPE65 cause Leber's congenital amaurosis and other forms of autosomal recessive retinitis pigmentosa which are associated with early-onset blindness. Structural highlights The inhibitor RPE65 drug '''emixustat''' binds in the active site near the enzyme's membrane-embedded substrate-entry point. The catalytic Fe+2 is 5-coordinated.

3D structures of retinoid isomerohydrolase

Updated on 12-October-2023

4f2z, 4f30, 4f3a, 4f3d, 3kvc, 3fsn – bRPE65 - bovine
4ryx, 4ryy, 4ryz, 4rsc – bRPE65 + emixustat
7k88 – bRPE65 + emixustat derivative
7k89, 7l0e – bRPE65 + emixustat derivative + palmitate
4zhk, 4rse – bRPE65 + MB-200
8doc - bRPE65 + MB-003 + palmitate
5ul5, 7k8g - bRPE65 + MB-004 + palmitate
5ulg - bRPE65 + MB-008 + palmitate

Proteopedia Page Contributors and Editors (what is this?)

Michal Harel, Alexander Berchansky, Joel L. Sussman