Retinal Pigment Epithelium-Specific Protein 65kDa (RPE65) ELISA Kits

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 Antibodies (75) and RPE65 Proteins (5) and many more products for this protein.

list all ELISA KIts Gene Name GeneID UniProt
RPE65 6121 Q16518
RPE65 19892  
RPE65 89826  
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Top RPE65 ELISA Kits at antibodies-online.com

Showing 6 out of 12 products:

Catalog No. Reactivity Sensitivity Range Images Quantity Supplier Delivery Price Details
Human 32 pg/mL 78-5000 pg/mL   96 Tests Log in to see 2 to 3 Days
$779.90
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Rat
  96 Tests Log in to see 11 to 18 Days
$589.86
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Mouse
  96 Tests Log in to see 11 to 18 Days
$589.86
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Cow
  96 Tests Log in to see 15 to 18 Days
$1,029.60
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Dog
  96 Tests Log in to see 15 to 18 Days
$1,095.60
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Chicken
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$1,095.60
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More ELISA Kits for RPE65 Interaction Partners

Human Retinal Pigment Epithelium-Specific Protein 65kDa (RPE65) interaction partners

  1. Early onset flecked retinal dystrophy associated with new compound heterozygous RPE65 variants in two unrelated Japanese patients.

  2. Autosomal dominant retinal dystrophy resembling choroideremia 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.

  3. By using whole-exome sequencing analysis, three RPE65 mutations were identified in two Japanese patients with leber congenital amaurosis (LCA). This approach would be useful for identification of disease-causing mutations of LCA.

  4. RPE65 variants are the most prevalent causes of Leber congenital amaurosis in Denmark.

  5. 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.

  6. 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.

  7. 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.

  8. three Leber congenital amaurosis -associated RPE65 mutants (R91W, Y249C and R515W) undergo rapid proteasomal degradation mediated by the 26 S proteasome non-ATPase regulatory subunit 13.

  9. 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.

  10. Data show that 4-phenylbutyrate (PBA) displayed a significant synergistic effect on the low temperature-mediated rescue of the mutant isomerase activity of RPE65.

  11. Expressions of MDSC, FOXP3+TILs, and CTLA-4 are relative stable after nCRT

  12. We showed that miR-410 directly regulates predicted target genes OTX2 and RPE65.

  13. Studies indicate that patients consistently reported improvement in their vision following delivery of recombinant adenoassociated virus (rAAV) that carried retinal pigment epithelium 65 protein (REP65) gene.

  14. All RPE65-mutant observers have consistent and substantial losses in temporal acuity and sensitivity compared with normal observers.

  15. when an amino-terminal fragment (Met(1)-Arg(33)) of the N170K/K297G double mutant of hRPE65 was replaced with the corresponding cRPE65 fragment, the isomerohydrolase activity was further increased to a level similar to that of cRPE65.

  16. We identified a novel LCA-related homozygous RPE65 mutation associated with a severe clinical presentation including an early and severe cone dysfunction.

  17. properties of disease causing RPE65 with regard to molecular pathogenic mechanism

  18. These results strongly suggest that causal mutations in RPE65 are responsible for retinal dystrophy in the affected individuals of consanguineous Pakistani families.

  19. These results indicate that the non-viral delivery of hRPE65 vectors can result in persistent, therapeutically efficacious gene expression in the retinal pigment epithelium .

  20. the RPE65-LCA patients had higher variability in kinetic field extent. VA variability in RPE65-LCA fell within reported results for retinitis pigmentosa.

Mouse (Murine) Retinal Pigment Epithelium-Specific Protein 65kDa (RPE65) interaction partners

  1. These results indicate that a toxic gain-of-function induced by the D477G RPE65 substitution may play a role in the pathogenesis of this form of dominant retinitis pigmentosa

  2. The present study describes identification of CU239, a novel non-retinoid inhibitor of RPE65, a key enzyme in the visual cycle. Our data demonstrated that CU239 selectively inhibited isomerase activity of RPE65, with IC50 of 6muM.

  3. 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.

  4. The loss of ERK1/2 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 cells, ultimately leading to retinal degeneration.

  5. these observations suggest that D477G acts as a dominant-negative mutant of RPE65 that delays chromophore regeneration.

  6. 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).

  7. 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.

  8. the RPE65 protein expression was abnormal

  9. 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.

  10. properties of disease causing RPE65 with regard to molecular pathogenic mechanism

  11. 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.

  12. Despite the previously reported upregulation of Cspg5 during retinal degeneration in Rpe65/ mice, no protective effect or any involvement of Cspg5 in disease progression was identified.

  13. To recapitulate this event in vivo, we examined tumor formation in NOG mice after subcutaneous injection of iPSCs with or without an iPSC-derived RPE sheet (2.5 x 10(5) RPE cells).

  14. Cone-opsin trafficking defects were replicated in Rpe65-/- Rho-/- retina-retinal pigment epithelium cultures.

  15. The Rpe65KO and tvrm148 mutations do not complement one another, proving that the tvrm148 mutation occurs in the Rpe65 locus and is the causative lesion driving visual function loss.

  16. Aromatic residues in the substrate cleft of RPE65 protein govern retinol isomerization and modulate its progression.

  17. a role for MYO7A in the translocation of RPE65

  18. Rpe65 deficiency may have many metabolic consequences in the underlying neuroretina. Glb1l3 was the only Glb-related member strongly downregulated in Rpe65(-/-) retinas before the onset and during progression of disease.

  19. Mef2c was the only Mef2 member markedly downregulated during retinal degeneration in Rpe65(-/-) mice.

  20. These results demonstrate that RPE65 within cones may be essential for the efficient regeneration of cone photopigments under bright-light conditions.

Fruit Fly (Drosophila melanogaster) Retinal Pigment Epithelium-Specific Protein 65kDa (RPE65) interaction partners

  1. study identifies NinaB as a key component for visual pigment production and provides evidence that chromophore in opsin-deficient photoreceptors can elicit retinal degeneration

  2. Beta-carotene is transported to the adult brain, where cellular uptake by NinaD allows cleavage by the NinaB enzyme to produce retinal

  3. Data show that carotenoid-isomerooxygenase activity of NinaB is more generally found in insects, and provide physiological evidence that carotenoids such as 11-cis-retinal can promote visual pigment biogenesis in the dark.

Cow (Bovine) Retinal Pigment Epithelium-Specific Protein 65kDa (RPE65) interaction partners

  1. Data indicate that Structural differences between delipidated and nondelipidated RPE65 uncovered key residues involved in substrate uptake and processing.

  2. oxidative stress during the visual cycle results in cleavage of RPE65

  3. the enzymatic activity of native RPE65 strongly depends on its membrane binding and phospholipid environment

  4. Rpe65 isomerase associates with membranes through an electrostatic interaction with acidic phospholipid headgroups.

  5. retinoid binding role for RPE65

  6. 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.

  7. Rpe65 converts an all-trans-retinyl ester to 11-cis-retinol and has been identified as retinoid isomerase. Mutations in RPE65 are associated with Leber hereditary optic atrophy

  8. Data show that RPE65 is a moderately specific retinoid binding protein directed at long chain all-trans-retinyl esters.

  9. investigation of metal ions required for the isomerohydrolase activity of RPE65 using the conversion of all-trans-[3H]retinol to 11-cis-[3H]retinol as the measure for isomerohydrolase activity

  10. LRAT is not required for isomerase activity beyond synthesis of retinyl-ester substrate, and the association of Rpe65 with membranes is neither dependent upon LRAT nor the result of S-palmitoylation

  11. RPE65 from the cone-dominant chicken RPE possesses significantly higher specific retinol isomerohydrolase activity, when compared with RPE65 from rod-dominant species

Zebrafish Retinal Pigment Epithelium-Specific Protein 65kDa (RPE65) interaction partners

  1. these results showed that residue 58 is a primary determinant of isomerization specificity, because the Y58N mutation in RPE65c and its reciprocal N58Y mutation in 13cIMH completely reversed the respective enzyme isomerization product specificities.

RPE65 Antigen Profile

Antigen 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 names and symbols associated with RPE65

  • RPE65, retinoid isomerohydrolase (RPE65) antibody
  • retinal pigment epithelium 65 (Rpe65) antibody
  • neither inactivation nor afterpotential B (ninaB) antibody
  • RPE65, retinoid isomerohydrolase (Rpe65) antibody
  • retinal pigment epithelium-specific protein 65kDa S homeolog (rpe65.S) antibody
  • retinal pigment epithelium-specific protein 65kDa L homeolog (rpe65.L) antibody
  • RPE65 homolog (rpe65) antibody
  • retinal pigment epithelium-specific protein 65kDa (rpe65) antibody
  • retinal pigment epithelium-specific protein 65c (rpe65c) antibody
  • retinal pigment epithelium-specific protein 65a (rpe65a) antibody
  • 65kDa antibody
  • A930029L06Rik antibody
  • anon-WO0148162.1 antibody
  • anon-WO0148163.1 antibody
  • beta-diox antibody
  • CG9347 antibody
  • DmelCG9347 antibody
  • DRPE65 antibody
  • LCA2 antibody
  • MGC52887 antibody
  • MGC85437 antibody
  • MGC154296 antibody
  • Mord1 antibody
  • mRPE65 antibody
  • rd12 antibody
  • RP20 antibody
  • RPE56a antibody
  • RPE56c antibody
  • rpe65 antibody
  • rpepa antibody
  • si:ch211-198n5.12 antibody
  • sRPE65 antibody
  • zgc:73213 antibody

Protein level used designations for RPE65

RBP-binding membrane protein , all-trans-retinyl-palmitate hydrolase , p63 , 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 , CG9347-PA , ninaB-PA , 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 , All-trans-retinylester 11-cis isomerohydrolase A , Retinal pigment epithelium-specific 65 kDa protein homolog A , all-trans-retinylester 11-cis isomerohydrolase A , retinal pigment epithelium 65a , retinal pigment epithelium-specific 65 kDa protein homolog A , retinal pigment epithelium-specific protein a

GENE ID SPECIES
6121 Homo sapiens
19892 Mus musculus
41678 Drosophila melanogaster
89826 Rattus norvegicus
282043 Bos taurus
379961 Xenopus laevis
395700 Gallus gallus
403803 Canis lupus familiaris
447613 Xenopus laevis
701764 Macaca mulatta
744567 Pan troglodytes
778842 Ciona intestinalis
100145692 Xenopus (Silurana) tropicalis
100397525 Callithrix jacchus
100453879 Pongo abelii
100516743 Sus scrofa
100588685 Nomascus leucogenys
100004076 Danio rerio
100219959 Taeniopygia guttata
100352270 Oryctolagus cuniculus
100721944 Cavia porcellus
393724 Danio rerio
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