Special Offers
100% Performance Guaranteed
| Description | |
|---|---|
| Catalogue Number | MAB5428-25UG |
| Brand Family | Chemicon® |
| Trade Name |
|
| Description | Anti-Retinal Pigment Epithelium 65 |
| Alternate Names |
|
| Product Information | |
|---|---|
| Format | Purified |
| Control |
|
| Presentation | Purified immunoglobulin. Liquid in 0.02M phosphate buffer, pH 7.6, 0.25M NaCl, and 0.1% sodium azide. |
| Quality Level | MQ100 |
| Applications | |
|---|---|
| Application | Anti-Retinal Pigment Epithelium 65 Antibody detects level of Retinal Pigment Epithelium 65 & has been published & validated for use in ELISA, IH, IP & WB. |
| Key Applications |
|
| Application Notes | Western blot: 1:5,000-1:20,000 on bovine RPE membranes using ECL. Suggested dilution buffer is TBS containing 10% calf serum, 0.25% T-20, 1M D-glucose with 10% glycerol. Suggested blocking buffer is TBS containing 2% BSA and 0.5% Tween 20. Preferred gel percentage is 10%. Immunohistochemistry on frozen tissue sections: 1:250-1:500 Immunoprecipitation: 20 μg of antibody in a reaction volume of 500 μL. Immunoaffinity purification ELISA Optimal working dilutions must be determined by end user. |
| Biological Information | |
|---|---|
| Immunogen | Bovine RPE microsomal membranes. |
| Clone | 401.8B11.3D9 |
| Concentration | Please refer to lot specific datasheet. |
| Host | Mouse |
| Specificity | Reacts with Retinal Pigment Epithelium 65 (RPE65). On bovine RPE membranes the antibody recognizes a protein with a molecular weight of ~65 kDa. |
| Isotype | IgG |
| Species Reactivity |
|
| Antibody Type | Monoclonal Antibody |
| Entrez Gene Number |
|
| Entrez Gene Summary | This gene encodes a protein which is located in the retinal pigment epithelium and is involved in the production of 11-cis retinal and in visual pigment regeneration. There are two forms of this protein, a soluble form called sRPE65, and a palmitoylated, membrane-bound form known as mRPE65. mRPE65 serves as the palmitoyl donor for lecithin retinol acyl transferase (LRAT), the enzyme that catalyzes the vitamin A to all trans retinol step of the chromophore regeneration process. Both mRPE65 and sRPE65 also serve as regulatory proteins, with the ratio and concentrations of these molecules playing a role in the inhibition of 11-cis retinal synthesis. Mutations in this gene have been associated with Leber congenital amaurosis type 2 (LCA2) and retinitis pigmentosa. |
| Gene Symbol |
|
| Purification Method | Protein A Purfied |
| UniProt Number |
|
| UniProt Summary | FUNCTION: SwissProt: Q16518 # Plays 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 (By similarity). SIZE: 533 amino acids; 60948 Da SUBCELLULAR LOCATION: Cytoplasm (By similarity). Cell membrane; Lipid-anchor (By similarity). Note=Attached to the membrane by a lipid anchor when palmitoylated (membrane form), soluble when unpalmitoylated (By similarity). TISSUE SPECIFICITY: Retinal pigment epithelium specific. PTM: Palmitoylation 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) (By similarity). DISEASE: SwissProt: Q16518 # Defects 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:180069]. 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. SIMILARITY: SwissProt: Q16518 ## Belongs to the carotenoid oxygenase family. |
| Molecular Weight | ~ 65 kDa |
| Product Usage Statements | |
|---|---|
| Quality Assurance | Tested |
| Usage Statement |
|
| Storage and Shipping Information | |
|---|---|
| Storage Conditions | Maintain for 1 year at 2–8°C from date of shipment. Aliquot to avoid repeated freezing and thawing. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap. |
| Packaging Information | |
|---|---|
| Material Size | 25 μg |