raised against RPE microsomal membranes of bovine origin.
Key Feature
Clonality
Monoclonal
Isotype
IgG1, k
Host Species
Mouse
Tested Applications
WB
WB:1:100-1:1000
Species Reactivity
HumanMouseRat
Concentration
0.1mg/ml
Purification
Affinity purified
Target Information
Gene Symbol
RPE65
Gene Synonyms
p63 BCO3 LCA2 RP20 rd12 mRPE65 sRPE65
Gene Full Name
retinoid isomerohydrolase RPE65
Gene Summary
The protein encoded by this gene is a component of the vitamin A visual cycle of the retina which supplies the 11-cis retinal chromophore of the photoreceptors opsin visual pigments. It is a member of the carotenoid cleavage oxygenase superfamily. All members of this superfamily are non-heme iron oxygenases with a seven-bladed propeller fold and oxidatively cleave carotenoid carbon:carbon double bonds. However, the protein encoded by this gene has acquired a divergent function that involves the concerted O-alkyl ester cleavage of its all-trans retinyl ester substrate and all-trans to 11-cis double bond isomerization of the retinyl moiety. As such, it performs the essential enzymatic isomerization step in the synthesis of 11-cis retinal. Mutations in this gene are associated with early-onset severe blinding disorders such as Leber congenital. [provided by RefSeq, Oct 2017]