Key features and details
- Rabbit monoclonal [EPR7024(N)] to RPE65 - BSA and Azide free
- Suitable for: WB, IP
- Reacts with: Mouse, Rat, Human
Product nameAnti-RPE65 antibody [EPR7024(N)] - BSA and Azide free
See all RPE65 primary antibodies
DescriptionRabbit monoclonal [EPR7024(N)] to RPE65 - BSA and Azide free
Tested applicationsSuitable for: WB, IPmore details
Unsuitable for: ICC/IF or IHC-P
Species reactivityReacts with: Mouse, Rat, Human
Synthetic peptide within Human RPE65 aa 450 to the C-terminus (Cysteine residue). The exact sequence is proprietary.
Database link: Q16518
Ab249901 is the carrier-free version of ab175936. This format is designed for use in antibody labeling, including fluorochromes, metal isotopes, oligonucleotides, enzymes.
ab249901 is compatible with the Maxpar® Antibody Labeling Kit from Fluidigm.
Maxpar® is a trademark of Fluidigm Canada Inc.
This product is a recombinant monoclonal antibody, which offers several advantages including:
- - High batch-to-batch consistency and reproducibility
- - Improved sensitivity and specificity
- - Long-term security of supply
- - Animal-free production
Storage instructionsShipped at 4°C. Store at +4°C. Do Not Freeze.
Storage bufferpH: 7.2
Concentration information loading...
Our Abpromise guarantee covers the use of ab249901 in the following tested applications.
|WB||Use at an assay dependent concentration. Predicted molecular weight: 61 kDa.|
|IP||Use at an assay dependent concentration.|
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 [EPR7024(N)] - C-terminal (ab175936) at 1/1000 dilution
Lane 1 : Mouse eyeball lysate
Lane 2 : Rat eyeball lysate
Lane 3 : Y79 lysate
Lysates/proteins at 10 µg per lane.
Predicted band size: 61 kDa
This data was developed using ab175936, the same antibody clone in a different buffer formulation.
ab249901 has not yet been referenced specifically in any publications.