Key features and details
- Mouse monoclonal [401.8B11.3D9] to RPE65
- Suitable for: WB, IHC-Fr
- Reacts with: Mouse, Cow, Human, Pig
- Isotype: IgG1
Product nameAnti-RPE65 antibody [401.8B11.3D9]
See all RPE65 primary antibodies
DescriptionMouse monoclonal [401.8B11.3D9] to RPE65
Tested applicationsSuitable for: WB, IHC-Frmore details
Species reactivityReacts with: Mouse, Cow, Human, Pig
Predicted to work with: Dog
Tissue, cells or virus corresponding to RPE65. Bovine RPE microsomal membrane proteins.
- WB: Recombinant human RPE protein expressed in COS-7 cells and bovine RPE membrane fraction. IHC-Fr: Mouse retina tissue. ICC/IF: ARPE19 cells. Flow Cytometry: Mouse retina and choroid cells.
Expression in primary culture of RPE cells is undetectable in Western blot after day 14. Established cell lines are similarly devoid of RPE65 protein, as indicated in western blot. However, mRNA levels are detectable by Northern for at least 7 weeks. (see reference Hamel, C.P., et al. Molecular Cloning and expression of RPE65 a novel retinal pigment epithelium-specific microsomal protein that is post-transcriptionally regulated in vitro. JBC. 268(21): 15751-15757, 1993.)
Storage instructionsShipped at 4°C. Upon delivery aliquot and store at -20°C. Avoid freeze / thaw cycles.
Storage bufferpH: 7.40
Preservative: 0.05% Sodium azide
Concentration information loading...
Purification notesPurified from ascites.
Light chain typekappa
Our Abpromise guarantee covers the use of ab13826 in the following tested applications.
The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.
|WB||1/5000 - 1/10000. Detects a band of approximately 65 kDa.|
FunctionPlays important roles in the production of 11-cis retinal and in visual pigment regeneration. The soluble form binds vitamin A (all-trans-retinol), making it available for LRAT processing to all-trans-retinyl ester. The membrane form, palmitoylated by LRAT, binds all-trans-retinyl esters, making them available for IMH (isomerohydrolase) processing to all-cis-retinol. The soluble form is regenerated by transferring its palmitoyl groups onto 11-cis-retinol, a reaction catalyzed by LRAT. The enzymatic activity is linearly dependent of the expression levels and membrane association.
Tissue specificityRetinal pigment epithelium specific.
Involvement in diseaseDefects in RPE65 are the cause of Leber congenital amaurosis type 2 (LCA2) [MIM:204100]. LCA designates a clinically and genetically heterogeneous group of childhood retinal degenerations, generally inherited in an autosomal recessive manner. Affected infants have little or no retinal photoreceptor function as tested by electroretinography. LCA represents the most common genetic cause of congenital visual impairment in infants and children.
Defects in RPE65 are the cause of retinitis pigmentosa type 20 (RP20) [MIM:613794]. RP leads to degeneration of retinal photoreceptor cells. Patients typically have night vision blindness and loss of midperipheral visual field. As their condition progresses, they lose their far peripheral visual field and eventually central vision as well. RP20 inheritance is autosomal dominant.
Sequence similaritiesBelongs to the carotenoid oxygenase family.
modificationsPalmitoylation by LRAT regulates ligand binding specificity; the palmitoylated form (membrane form) specifically binds all-trans-retinyl-palmitate, while the soluble unpalmitoylated form binds all-trans-retinol (vitamin A).
Cellular localizationCytoplasm. Cell membrane. Attached to the membrane by a lipid anchor when palmitoylated (membrane form), soluble when unpalmitoylated.
- Information by UniProt
- All-trans-retinyl-palmitate hydrolase antibody
- LCA 2 antibody
- LCA2 antibody
All lanes : Anti-RPE65 antibody [401.8B11.3D9] (ab13826) at 1/5000 dilution
Lane 1 : Recombinant human RPE-transfected COS-7 (African green monkey kidney fibroblast-like cell line) cell lysate at 20 µg
Lane 2 : Bovine retinal pigment epithelium membrane fraction at 5 µg
All lanes : Alkaline phosphatase conjugated goat-anti Mouse IgG
Observed band size: 60 kDa why is the actual band size different from the predicted?
Frozen sectioned mouse retina tissue stained for RPE65 with ab13826 at a 1/250 dilution in immunohistochemical analysis.
ab13826 has been referenced in 40 publications.
- Findlay AS et al. Fam151b, the mouse homologue of C.elegans menorin gene, is essential for retinal function. Sci Rep 10:437 (2020). PubMed: 31949211
- Vidal-Gil L et al. Poly ADP ribosylation and extracellular vesicle activity in rod photoreceptor degeneration. Sci Rep 9:3758 (2019). PubMed: 30842506
- Matsumoto E et al. Fabricating retinal pigment epithelial cell sheets derived from human induced pluripotent stem cells in an automated closed culture system for regenerative medicine. PLoS One 14:e0212369 (2019). PubMed: 30865653
- Milenkovic A et al. The Y227N mutation affects bestrophin-1 protein stability and impairs sperm function in a mouse model of Best vitelliform macular dystrophy. Biol Open 8:N/A (2019). PubMed: 31201163
- Guo Y et al. Modeling Retinitis Pigmentosa: Retinal Organoids Generated From the iPSCs of a Patient With the USH2A Mutation Show Early Developmental Abnormalities. Front Cell Neurosci 13:361 (2019). PubMed: 31481876