Retinoid isomerohydrolase

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Revision as of 10:17, 28 December 2016

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spinqualitylabelsall models Caption for this structure Show:Asymmetric Unit Biological Assembly Drag the structure with the mouse to rotate   Export Animated Image 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[1]. The RPE65 inhibitor amixustat is used as a drug which modulates visual cycle activity in age-related macular degeneration[2]. 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[3]. Structural highlights

3D structures of resolvase

Updated on 28-December-2016

4f2z, 4f30, 4f3a, 4f3d, 3kvc, 3fsn – bRPE65 - bovine
4ryx, 4ryy, 4ryz, 4rsc – bRPE65 + emixustat
4zhk, 4rse – bRPE65 + MB-200

References

1. ↑ Moiseyev G, Takahashi Y, Chen Y, Gentleman S, Redmond TM, Crouch RK, Ma JX. RPE65 is an iron(II)-dependent isomerohydrolase in the retinoid visual cycle. J Biol Chem. 2006 Feb 3;281(5):2835-40. Epub 2005 Nov 30. PMID:16319067 doi:http://dx.doi.org/10.1074/jbc.M508903200
2. ↑ Zhang J, Kiser PD, Badiee M, Palczewska G, Dong Z, Golczak M, Tochtrop GP, Palczewski K. Molecular pharmacodynamics of emixustat in protection against retinal degeneration. J Clin Invest. 2015 Jul 1;125(7):2781-94. doi: 10.1172/JCI80950. Epub 2015 Jun, 15. PMID:26075817 doi:http://dx.doi.org/10.1172/JCI80950
3. ↑ Cai X, Conley SM, Naash MI. RPE65: role in the visual cycle, human retinal disease, and gene therapy. Ophthalmic Genet. 2009 Jun;30(2):57-62. doi: 10.1080/13816810802626399. PMID:19373675 doi:http://dx.doi.org/10.1080/13816810802626399