重组65kDa视网膜色素上皮细胞特异性蛋白(RPE65)

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重组视网膜色素上皮细胞特异性蛋白65
Recombinant RPE65

基因名：

RPE65

产品别名：

BCO3； LCA2； RP20； mRPE65； p63； rd12； sRPE65； RPE65； retinoid isomerohydrolase RPE65； retinoid isomerohydrolase RPE65； retinoid isomerohydrolase； BCO family, member 3； RBP-binding membrane protein； RPE65, retinoid isomerohydrolase； all-trans-retinyl-palmitate hydrolase； lutein isomerase； meso-zeaxanthin isomerase； retinal pigment epithelium specific protein 65； retinal pigment epithelium-specific 65 kDa protein； retinal pigment epithelium-specific protein 65kDa； retinitis pigmentosa 20 (autosomal recessive)； retinol isomerase； 65kDa视网膜色素上皮细胞特异性蛋白(RPE65)；视网膜色素上皮细胞特异性蛋白65；

背景信息：

The retinal pigment epithelium (RPE) is a monolayer simple epithelium in proximity to the outer surface of the retinal photoreceptor cells. Retinal pigment epithelium-specific protein (RPE65) is a 65 kDa protein belonging to the β-carotene dioxygenase family. This protein is important in 11-cis retinal production as well as in visual pigment regeneration. RPE65 is attached to the membrane by a lipid anchor when palmitoylated (membrane form) and soluble when unpalmitoylated. The soluble form of the protein binds vitamin A. Defects in RPE65 causes autosomal dominant retinitis pigmentosa and/or Leber congenital amaurosis type 2.