Retinal Pigment Epithelium Specific Protein (Retinal Pigment Epithelium-specific 65kD Protein, RPE65, All-trans-retinyl-palmitate Hydrolase, LCA2, mRPE65, rd12, Retinol Isomerase, Retinoid Isomerohydrolase, RP20, sRPE65) (Biotin)

Catalogue Number: R1659-70B-USB

Manufacturer:United States Biological
Type:Monoclonal Primary Antibody - Conjugated
Shipping Condition:Blue Ice
Unit(s): 200 ul
Host name: Mouse
Clone: 8B99 (401.8B11.3D9)
Isotype: IgG1, kappa
Immunogen: Bovine RPE microsomal membrane proteins. Cellular Localization: Cytoplasmic, microsomeal and cell membrane. Species Sequence Homology: birds; 89%, zebrafish; 74%, sea lamprey; 71%
Application: ICC, WB, IHC

Description

Description: Highly conserved among vertebrate species, RPE65 is a major protein of the retinal pigment epithelium (RPE). RPE65 is essential for the regeneration of rhodopsin in the visual cycle. Mutations in RPE65 are responsible for certain forms of autosomal recessive severe retinal dystrophy, including Leber Congenital Amaurosis (LCA). Loss of function results in a block in retinoid processing required for the synthesis of 11-cis retinal and the accumulation of retinyl esters in the RPE.

Additional Text

Purification

Protein A purified

Antibody Clonality

Monoclonal