RPE65 Antibody (401.8B11.3D9) [Alexa Fluor® 488]

Novus Biologicals, part of Bio-Techne | Catalog # NB100-355AF488

Novus Biologicals, part of Bio-Techne

Key Product Details

Species Reactivity

Validated:

Human, Mouse, Rat, Porcine, Bovine, Canine, Chicken, Primate, Xenopus

Cited:

Human, Mouse

Applications

Validated:

CyTOF-ready, Flow Cytometry, Immunocytochemistry/ Immunofluorescence, Immunofluorescence, Immunohistochemistry, Immunohistochemistry-Frozen, Immunohistochemistry-Paraffin, Immunoprecipitation, In vivo assay, Knockout Validated, Simple Western, Western Blot

Cited:

IF/IHC, Immunohistochemistry-Paraffin

Label

Alexa Fluor 488 (Excitation = 488 nm, Emission = 515-545 nm)

Antibody Source

Monoclonal Mouse IgG1 kappa Clone # 401.8B11.3D9

Concentration

Please see the vial label for concentration. If unlisted please contact technical services.

View all RPE65 Antibodies »

Product Specifications

Immunogen

This RPE65 Antibody (401.8B11.3D9) was developed against bovine RPE65 microsomal membrane proteins. [UniProt# Q28175]

Reactivity Notes

Primate reactivity reported in scientific literature (PMID: 31660416).

Localization

Cytoplasmic, Microsomeal and Cell Membrane

Marker

Retinal Pigment Epithelium Marker

Clonality

Monoclonal

Host

Mouse

Isotype

IgG1 kappa

Scientific Data Images for RPE65 Antibody (401.8B11.3D9) [Alexa Fluor® 488]

Product Image: RPE65 Antibody (401.8B11.3D9) [Alexa Fluor® 488] [NB100-355AF488] - Vial of Alexa Fluor 488 conjugated antibody. Alexa Fluor 488 is optimally excited at 490 nm by the Blue laser (488 nm) and has an emission maximum of 525 nm.

Applications for RPE65 Antibody (401.8B11.3D9) [Alexa Fluor® 488]

Application
Recommended Usage

CyTOF-ready

Optimal dilutions of this antibody should be experimentally determined.

Flow Cytometry

Optimal dilutions of this antibody should be experimentally determined.

Immunocytochemistry/ Immunofluorescence

Optimal dilutions of this antibody should be experimentally determined.

Immunofluorescence

Optimal dilutions of this antibody should be experimentally determined.

Immunohistochemistry

Optimal dilutions of this antibody should be experimentally determined.

Immunohistochemistry-Frozen

Optimal dilutions of this antibody should be experimentally determined.

Immunohistochemistry-Paraffin

Optimal dilutions of this antibody should be experimentally determined.

Immunoprecipitation

Optimal dilutions of this antibody should be experimentally determined.

In vivo assay

Optimal dilutions of this antibody should be experimentally determined.

Knockout Validated

Optimal dilutions of this antibody should be experimentally determined.

Simple Western

Optimal dilutions of this antibody should be experimentally determined.

Western Blot

Optimal dilutions of this antibody should be experimentally determined.
Application Notes
Optimal dilution of this antibody should be experimentally determined.
Please Note: Optimal dilutions of this antibody should be experimentally determined.

Formulation, Preparation, and Storage

Purification

Protein G purified

Formulation

50mM Sodium Borate

Preservative

0.05% Sodium Azide

Concentration

Please see the vial label for concentration. If unlisted please contact technical services.

Shipping

The product is shipped with polar packs. Upon receipt, store it immediately at the temperature recommended below.

Stability & Storage

Store at 4C in the dark.

Background: RPE65

RPE65 (retinal pigment epithelium-specific 65 kDa protein) was first described in 1991 and is a key isomerase in RPE that is largely localized to the smooth endoplasmic reticulum (1,2). The RPE65 gene is located on chromosome 1p31 and the protein is synthesized as 533 amino acids in length with a theoretical molecular weight of 61 kDa, although appearing near 65 kDa in SDS-PAGE (1,3). RPE65 serves an important enzymatic function in the visual cycle in converting all-trans-retinyl esters into 11-cis-retinal (1-6). In the visual cycle, light activates rhodopsin and other visual pigments in the photoreceptor cells (rods and cones), eventually generating all-trans-retinol which is transported to the RPE and converted to the vitamin A-derived chromophore 11-cis-rentinal via RPE65 and is then diffused back to the photoreceptor to continue the cycle (1-6). Although it is still unclear, some studies have suggested that S-palmitoylation post-translational modification of RPE65 is responsible for the stability and anchoring to the RPE membrane and therefore functioning enzymatic activity (1,2).

Given its essential role in the vision cycle, it is understandable that mutations in RPE65 are associated with a variety of inherited retinal dystrophies (1, 3-6). Leber Congenital Amaurosis (LCA) and retinitis pigmentosa (RP) are two of the most common retinal dystrophies associated with bi-allelic RPE65 gene mutations (5,6). In 2017 the FDA approved an in vivo gene therapy for treatment of RPE65-associated diseases (5,6). The drug Voretigene Neparvovec, also called Luxturna, is delivered sub-retinally and transduces RPE cells with cDNA encoding for normal RPE65 to help restore vision (5,6). There are several promising completed and ongoing clinical trials for treating RPE65-associated diseases using gene replacement therapy (5).

References

1. Kiser, P. D., & Palczewski, K. (2010). Membrane-binding and enzymatic properties of RPE65. Progress in retinal and eye research. https://doi.org/10.1016/j.preteyeres.2010.03.002

2. Uppal, S., Poliakov, E., Gentleman, S., & Redmond, T. M. (2019). RPE65 Palmitoylation: A Tale of Lipid Posttranslational Modification. Advances in experimental medicine and biology. https://doi.org/10.1007/978-3-030-27378-1_88

3. Redmond T. M. (2009). Focus on Molecules: RPE65, the visual cycle retinol isomerase. Experimental eye research. https://doi.org/10.1016/j.exer.2008.07.015

4. Saari J. C. (2016). Vitamin A and Vision. Sub-cellular biochemistry. https://doi.org/10.1007/978-94-024-0945-1_9

5. Miraldi Utz, V., Coussa, R. G., Antaki, F., & Traboulsi, E. I. (2018). Gene therapy for RPE65-related retinal disease. Ophthalmic genetics. https://doi.org/10.1080/13816810.2018.1533027

6. Apte R. S. (2018). Gene Therapy for Retinal Degeneration. Cell. https://doi.org/10.1016/j.cell.2018.03.021

Alternate Names

All-trans-retinyl-palmitate hydrolase, BCO family, member 3, BCO3, EC 3.1.1.64, EC:3.1.1.64, EC:5.3.3.22, LCA2, lutein isomerase, meso-zeaxanthin isomerase, mRPE65, p63, RBP-binding membrane protein, rd12, retinal pigment epithelium specific protein 65, Retinal pigment epithelium-specific 65 kDa protein, retinal pigment epithelium-specific protein 65kDa, retinitis pigmentosa 20 (autosomal recessive), retinoid isomerohydrolase, Retinol isomerase, RP20, RPE65, RPE65, retinoid isomerohydrolase, sRPE65

Gene Symbol

RPE65

Additional RPE65 Products

Product Documents for RPE65 Antibody (401.8B11.3D9) [Alexa Fluor® 488]

Product Datasheets

Certificate of Analysis

To download a Certificate of Analysis, please enter a lot number in the search box below.

Download SDS

Product Specific Notices for RPE65 Antibody (401.8B11.3D9) [Alexa Fluor® 488]

Alexa Fluor (R) products are provided under an intellectual property license from Life Technologies Corporation. The purchase of this product conveys to the buyer the non-transferable right to use the purchased product and components of the product only in research conducted by the buyer (whether the buyer is an academic or for-profit entity). The sale of this product is expressly conditioned on the buyer not using the product or its components, or any materials made using the product or its components, in any activity to generate revenue, which may include, but is not limited to use of the product or its components: (i) in manufacturing; (ii) to provide a service, information, or data in return for payment; (iii) for therapeutic, diagnostic or prophylactic purposes; or (iv) for resale, regardless of whether they are resold for use in research. For information on purchasing a license to this product for purposes other than as described above, contact Life Technologies Corporation, 5791 Van Allen Way, Carlsbad, CA 92008 USA or outlicensing@lifetech.com. This conjugate is made on demand. Actual recovery may vary from the stated volume of this product. The volume will be greater than or equal to the unit size stated on the datasheet.

This product is for research use only and is not approved for use in humans or in clinical diagnosis. Primary Antibodies are guaranteed for 1 year from date of receipt.

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