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

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. De plus, nous expédions RPE65 Anticorps (67) et RPE65 Protéines (6) et beaucoup plus de produits pour cette protéine.

list all ELISA KIts Gène GeneID UniProt
RPE65 6121 Q16518
RPE65 19892  
RPE65 89826  
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Humain 32 pg/mL 78-5000 pg/mL   96 Tests Connectez-vous pour afficher 2 to 3 Days
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Plus Kits ELISA pour RPE65 partenaires d'interaction

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

  1. Early onset flecked retinal dystrophy (Montrer MERTK Kits ELISA) associated with new compound heterozygous RPE65 variants in two unrelated Japanese patients.

  2. Autosomal dominant retinal dystrophy (Montrer MERTK Kits ELISA) 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.

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

  1. 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.

  2. 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.

  3. The loss of ERK1/2 (Montrer MAPK1/3 Kits ELISA) 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 (Montrer RPE Kits ELISA) cells, ultimately leading to retinal degeneration.

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

  5. 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).

  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. the RPE65 protein expression was abnormal

  8. 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.

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

  10. 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.

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 (Montrer RHO Kits ELISA)-deficient photoreceptors can elicit retinal degeneration

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

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

RPE65 profil antigène

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

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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