Rabbit RPE65 Polyclonal Antibody | anti-RPE65 antibody
RPE65 Antibody BIOTIN-Conjugated
IP: 1:200
LCA 2 antibody
Leber congenital amaurosis antibody
p63 antibody
rd 12 antibody
Retinal pigment epithelium specific 61 kDa protein antibody
Retinal pigment epithelium specific 65 kDa protein antibody
retinitis pigmentosa 20 antibody
Retinoid isomerohydrolase antibody
RP20 antibody
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.
NCBI and Uniprot Product Information
Uniprot Description
RPE65: 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. The enzymatic activity is linearly dependent of the expression levels and membrane association. Defects in RPE65 are the cause of Leber congenital amaurosis type 2 (LCA2). 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). 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. Defects in RPE65 are a cause of autosomal dominant retinitis pigmentosa with choroidal involvement (PubMed:21654732). Affected individuals show reduction of central vision, constriction of visual fields, night blindness and chorioretinal atrophy. Belongs to the carotenoid oxygenase family.
Protein type: EC 3.1.1.64; Cofactor and Vitamin Metabolism - retinol
Cellular Component: cytoplasm; endoplasmic reticulum; intracellular membrane-bound organelle; membrane; plasma membrane
Molecular Function: hydrolase activity; isomerase activity; metal ion binding; oxidoreductase activity, acting on single donors with incorporation of molecular oxygen, incorporation of two atoms of oxygen; retinal isomerase activity
Biological Process: detection of light stimulus involved in visual perception; insulin receptor signaling pathway; regulation of rhodopsin gene expression; response to stimulus; retina development in camera-type eye; retina morphogenesis in camera-type eye; retinal homeostasis; retinal metabolic process; retinol metabolic process; visual perception
Research Articles on RPE65
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Product Notes
The RPE65 rpe65 (Catalog #AAA544745) is an Antibody produced from Rabbit and is intended for research purposes only. The product is available for immediate purchase. The RPE65 Antibody BIOTIN-Conjugated reacts with Bovine, Human, Mouse, Rat and may cross-react with other species as described in the data sheet. AAA Biotech's RPE65 can be used in a range of immunoassay formats including, but not limited to, ELISA (EIA), Immunoprecipitation (IP), Western Blot (WB). Western Blot: 1:500 IP: 1:200. Researchers should empirically determine the suitability of the RPE65 rpe65 for an application not listed in the data sheet. Researchers commonly develop new applications and it is an integral, important part of the investigative research process. It is sometimes possible for the material contained within the vial of "RPE65, Polyclonal Antibody" to become dispersed throughout the inside of the vial, particularly around the seal of said vial, during shipment and storage. We always suggest centrifuging these vials to consolidate all of the liquid away from the lid and to the bottom of the vial prior to opening. Please be advised that certain products may require dry ice for shipping and that, if this is the case, an additional dry ice fee may also be required.Precautions
All products in the AAA Biotech catalog are strictly for research-use only, and are absolutely not suitable for use in any sort of medical, therapeutic, prophylactic, in-vivo, or diagnostic capacity. By purchasing a product from AAA Biotech, you are explicitly certifying that said products will be properly tested and used in line with industry standard. AAA Biotech and its authorized distribution partners reserve the right to refuse to fulfill any order if we have any indication that a purchaser may be intending to use a product outside of our accepted criteria.Disclaimer
Though we do strive to guarantee the information represented in this datasheet, AAA Biotech cannot be held responsible for any oversights or imprecisions. AAA Biotech reserves the right to adjust any aspect of this datasheet at any time and without notice. It is the responsibility of the customer to inform AAA Biotech of any product performance issues observed or experienced within 30 days of receipt of said product. To see additional details on this or any of our other policies, please see our Terms & Conditions page.Item has been added to Shopping Cart
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