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RPE65 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. Additionally we are shipping RPE65 Kits (11) and RPE65 Proteins (6) and many more products for this protein.
Showing 10 out of 65 products:
Chicken Monoclonal RPE65 Primary Antibody for CyTOF, FACS - ABIN151819
Jacobson, Aleman, Cideciyan, Heon, Golczak, Beltran, Sumaroka, Schwartz, Roman, Windsor, Wilson, Aguirre, Stone, Palczewski: Human cone photoreceptor dependence on RPE65 isomerase. in Proceedings of the National Academy of Sciences of the United States of America 2007
Show all 45 Pubmed References
Chicken Monoclonal RPE65 Primary Antibody for ICC, IF - ABIN314161
Jiang, Ke, Sun, Han, Kaplan, Shao: Reactivation of uveitogenic T cells by retinal astrocytes derived from experimental autoimmune uveitis-prone B10RIII mice. in Investigative ophthalmology & visual science 2008
Show all 10 Pubmed References
Chicken Monoclonal RPE65 Primary Antibody for IHC (fro), IHC (p) - ABIN314162
Lall, Ferrell, Nagar, Fleisher, McGahan: Iron regulates L-cystine uptake and glutathione levels in lens epithelial and retinal pigment epithelial cells by its effect on cytosolic aconitase. in Investigative ophthalmology & visual science 2008
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Rat (Rattus) Polyclonal RPE65 Primary Antibody for ELISA, WB - ABIN4351025
Rolling, Le Meur, Stieger, Smith, Weber, Deschamps, Nivard, Mendes-Madeira, Provost, Péréon, Cherel, Ali, Hamel, Moullier, Rolling et al.: Gene therapeutic prospects in early onset of severe retinal dystrophy: restoration of vision in RPE65 Briard dogs using an AAV serotype 4 vector that specifically targets the retinal pigmented ... in Bulletin et mémoires de l'Académie royale de médecine de Belgique 2007
Chicken Monoclonal RPE65 Primary Antibody for FACS, ICC - ABIN4351039
Lyzogubov, Bora, Wu, Horn, de Roque, Rudolf, Atkinson, Bora: The Complement Regulatory Protein CD46 Deficient Mouse Spontaneously Develops Dry-Type Age-Related Macular Degeneration-Like Phenotype. in The American journal of pathology 2016
Autosomal dominant retinal dystrophy (show MERTK Antibodies) resembling choroideremia (show CHM Antibodies) can arise from a heterozygous mutation in RPE65. It may manifest with mild disease or be non-penetrant. Awareness of these unusual presentations can facilitate targeted molecular investigation.
By using whole-exome sequencing analysis, three RPE65 mutations were identified in two Japanese patients with leber congenital amaurosis (LCA (show CLTA Antibodies)). This approach would be useful for identification of disease-causing mutations of LCA (show CLTA Antibodies).
RPE65 variants are the most prevalent causes of Leber congenital amaurosis in Denmark.
Hypomorphic mutations of RPE65 are associated with mild disease in childhood with preservation of good visual acuity into adulthood; they may in rare cases be associated with a flecked retina appearance similar to fundus albipunctatus.
Influx of T lymphocytes was associated with retinal pigment epithelium and choroidal thinning and diminished expression of RPE65 mRNA, an essential enzyme of the visual cycle.
These data also help define minimal requirements of chromophore for photoreceptor survival in vivo and may be useful in assessing a beneficial therapeutic dose for RPE65 gene therapy in humans.
three Leber congenital amaurosis -associated RPE65 mutants (R91W, Y249C and R515W) undergo rapid proteasomal degradation mediated by the 26 S proteasome non-ATPase (show DNAH8 Antibodies) regulatory subunit 13.
Studies indicate that patients with retinol isomerase RPE65R91W mutation have useful cone-mediated vision in the first decade of life, suggesting partial activity of the mutant RPE65R91W protein.
Data show that 4-phenylbutyrate (PBA) displayed a significant synergistic effect on the low temperature-mediated rescue of the mutant isomerase activity of RPE65.
Expressions of MDSC, FOXP3 (show FOXP3 Antibodies)+TILs, and CTLA-4 (show CTLA4 Antibodies) are relative stable after nCRT
Study describes a mouse model of Leber's Congenital Amaurosis type 2 with mutation in Rpe65 gene. Functional and biochemical studies confirm that vitamin A metabolism and visual processing are disrupted in this model.
The loss of ERK1/2 (show MAPK1/3 Antibodies) activity resulted in a significant decrease in the level of RPE65 expression, a decrease in ocular retinoid levels concomitant with low visual function, and a rapid disorganization of RPE (show RPE Antibodies) cells, ultimately leading to retinal degeneration.
these observations suggest that D477G acts as a dominant-negative mutant of RPE65 that delays chromophore regeneration.
The Leu450Met variant of RPE65 is expressed in C57BL/6 and in many genetically modified mice. It confers significant resistance to light induced retinal degeneration (LIRD).
the RPE65 protein expression was abnormal
properties of disease causing RPE65 with regard to molecular pathogenic mechanism
The rd12 lesion is in Rpe65. The rd12 mutant phenotype inherits in a semidominant manner. The effects of the mutant mRNA on visual function may result from inefficient binding to ribosomes for translation.
Despite the previously reported upregulation of Cspg5 (show CSPG5 Antibodies) during retinal degeneration in Rpe65/ mice, no protective effect or any involvement of Cspg5 (show CSPG5 Antibodies) in disease progression was identified.
study identifies NinaB as a key component for visual pigment production and provides evidence that chromophore in opsin (show RHO Antibodies)-deficient photoreceptors can elicit retinal degeneration
Data show that carotenoid-isomerooxygenase activity of NinaB is more generally found in insects, and provide physiological evidence that carotenoids such as 11-cis (show CISH Antibodies)-retinal can promote visual pigment biogenesis in the dark.
Data indicate that Structural differences between delipidated and nondelipidated RPE65 uncovered key residues involved in substrate uptake and processing.
oxidative stress during the visual cycle results in cleavage of RPE65
the enzymatic activity of native RPE65 strongly depends on its membrane binding and phospholipid environment
Rpe65 isomerase associates with membranes through an electrostatic interaction with acidic phospholipid headgroups.
retinoid binding role for RPE65
Quenching of protein fluorescence is used to demonstrate quantitatively that RPE65 functions by binding to and mobilizing the highly hydrophobic all-trans-retinyl esters, allowing them to enter the visual cycle.
Rpe65 converts an all-trans-retinyl ester to 11-cis (show CISH Antibodies)-retinol and has been identified as retinoid isomerase. Mutations in RPE65 are associated with Leber hereditary optic atrophy
Data show that RPE65 is a moderately specific retinoid binding protein directed at long chain all-trans-retinyl esters.
investigation of metal ions required for the isomerohydrolase activity of RPE65 using the conversion of all-trans-[3H]retinol to 11-cis (show CISH Antibodies)-[3H]retinol as the measure for isomerohydrolase activity
LRAT (show LRAT Antibodies) is not required for isomerase activity beyond synthesis of retinyl-ester substrate, and the association of Rpe65 with membranes is neither dependent upon LRAT (show LRAT Antibodies) nor the result of S-palmitoylation
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.
RBP-binding membrane protein
, all-trans-retinyl-palmitate hydrolase
, retinal pigment epithelium-specific 65 kDa protein
, retinitis pigmentosa 20 (autosomal recessive)
, retinoid isomerohydrolase
, retinol isomerase
, modifier of retinal degeneration 1
, retinal pigment epithelium, 65 kDa
, neither inactivation nor afterpotential B
, beta-carotene dioxygenase
, retinal pigment epithelium 65-like
, retinal pigment epithelium-specific protein (65kD)
, retinal pigment epithelium-specific protein 65kDa
, retinal pigment epithelium abundant protein RPE65
, rpe65 gene for retinal pigment epithelium-specific protein
, RPE65 homolog
, retinoid isomerohydrolase-like
, All-trans-retinyl ester 13-cis isomerohydrolase C
, All-trans-retinylester 11-cis isomerohydrolase C
, Retinal pigment epithelium-specific 65 kDa protein homolog C
, retinal Mueller cells isomerohydrolase