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RPE65 Antibody

  • 中文名稱:
    RPE65兔多克隆抗體
  • 貨號:
    CSB-PA624103LA01HU
  • 規格:
    ¥440
  • 圖片:
    • Western Blot
      Positive WB detected in: PC-3 whole cell lysate, MCF7 whole cell lysate, Rat Brain tissue lysate
      All lanes: RPE65 antibody at 1:1000
      Secondary
      Goat polyclonal to rabbit IgG at 1/50000 dilution
      Predicted band size: 61, 65 kDa
      Observed band size: 61 kDa
    • Immunofluorescence staining of Hela cell with CSB-PA624103LA01HU at 1:20, counter-stained with DAPI. The cells were fixed in 4% formaldehyde and blocked in 10% normal Goat Serum. The cells were then incubated with the antibody overnight at 4C. The secondary antibody was Alexa Fluor 488-congugated AffiniPure Goat Anti-Rabbit IgG(H+L).
    • Immunofluorescence staining of Hela cell with 5% goat serum, counter-stained with DAPI. The cells were fixed in 4% formaldehyde and blocked in 10% normal Goat Serum. The cells were then incubated with the antibody overnight at 4C. The secondary antibody was Alexa Fluor 488-congugated AffiniPure Goat Anti-Rabbit IgG(H+L).
    • Immunofluorescence staining of PC-3 cell with CSB-PA624103LA01HU at 1:20, counter-stained with DAPI. The cells were fixed in 4% formaldehyde and blocked in 10% normal Goat Serum. The cells were then incubated with the antibody overnight at 4C. The secondary antibody was Alexa Fluor 488-congugated AffiniPure Goat Anti-Rabbit IgG(H+L).
    • Immunofluorescence staining of PC-3 cell with 5% goat serum, counter-stained with DAPI. The cells were fixed in 4% formaldehyde and blocked in 10% normal Goat Serum. The cells were then incubated with the antibody overnight at 4C. The secondary antibody was Alexa Fluor 488-congugated AffiniPure Goat Anti-Rabbit IgG(H+L).
  • 其他:

產品詳情

  • 產品描述:

    The RPE65 polyclonal antibody is obtained from rabbits with the recombinant human RPE65 protein (348-484aa) immunized. It is specific for human and rat RPE65 proteins. This RPE65 antibody underwent protein G affinity and got at least 95% purity. The RPE65 antibody was validated for use in ELISA, WB, and IF applications.

    The RPE65 protein mainly acts as an isomerohydrolase enzyme that catalyzes the isomerization of all-trans retinyl esters to 11-cis retinol, a key step in the visual cycle essential for vision. Mutations in the RPE65 gene can result in a range of inherited retinal diseases, including Leber congenital amaurosis (LCA) and retinitis pigmentosa (RP).

  • 產品名稱:
    Rabbit anti-Homo sapiens (Human) RPE65 Polyclonal antibody
  • Uniprot No.:
  • 基因名:
  • 別名:
    All-trans-retinyl-palmitate hydrolase antibody; LCA 2 antibody; LCA2 antibody; Leber congenital amaurosis antibody; mRPE 65 antibody; mRPE65 antibody; p63 antibody; rd 12 antibody; rd12 antibody; Retinal pigment epithelium specific 61 kDa protein antibody; Retinal pigment epithelium specific 65 kDa protein antibody; Retinal pigment epithelium specific protein antibody; Retinal pigment epithelium specific protein 65kDa antibody; Retinal pigment epithelium-specific 65 kDa protein antibody; Retinitis pigmentosa 20 antibody; Retinoid isomerohydrolase antibody; Retinol isomerase antibody; RP 20 antibody; RP20 antibody; RPE 65 antibody; RPE65 antibody; RPE65_HUMAN antibody; sRPE 65 antibody; sRPE65 antibody
  • 宿主:
    Rabbit
  • 反應種屬:
    Human, Rat
  • 免疫原:
    Recombinant Human Retinoid isomerohydrolase protein (348-484AA)
  • 免疫原種屬:
    Homo sapiens (Human)
  • 標記方式:
    Non-conjugated

    本頁面中的產品,RPE65 Antibody (CSB-PA624103LA01HU),的標記方式是Non-conjugated。對于RPE65 Antibody,我們還提供其他標記。見下表:

    可提供標記
    標記方式 貨號 產品名稱 應用
    HRP CSB-PA624103LB01HU RPE65 Antibody, HRP conjugated ELISA
    FITC CSB-PA624103LC01HU RPE65 Antibody, FITC conjugated
    Biotin CSB-PA624103LD01HU RPE65 Antibody, Biotin conjugated ELISA
  • 克隆類型:
    Polyclonal
  • 抗體亞型:
    IgG
  • 純化方式:
    >95%, Protein G purified
  • 濃度:
    It differs from different batches. Please contact us to confirm it.
  • 保存緩沖液:
    Preservative: 0.03% Proclin 300
    Constituents: 50% Glycerol, 0.01M PBS, pH 7.4
  • 產品提供形式:
    Liquid
  • 應用范圍:
    ELISA, WB, IF
  • 推薦稀釋比:
    Application Recommended Dilution
    WB 1:500-1:5000
    IF 1:200-1:500
  • Protocols:
  • 儲存條件:
    Upon receipt, store at -20°C or -80°C. Avoid repeated freeze.
  • 貨期:
    Basically, we can dispatch the products out in 1-3 working days after receiving your orders. Delivery time maybe differs from different purchasing way or location, please kindly consult your local distributors for specific delivery time.
  • 用途:
    For Research Use Only. Not for use in diagnostic or therapeutic procedures.

產品評價

靶點詳情

  • 功能:
    Critical isomerohydrolase in the retinoid cycle involved in regeneration of 11-cis-retinal, the chromophore of rod and cone opsins. Catalyzes the cleavage and isomerization of all-trans-retinyl fatty acid esters to 11-cis-retinol which is further oxidized by 11-cis retinol dehydrogenase to 11-cis-retinal for use as visual chromophore. Essential for the production of 11-cis retinal for both rod and cone photoreceptors. Also capable of catalyzing the isomerization of lutein to meso-zeaxanthin an eye-specific carotenoid. 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.
  • 基因功能參考文獻:
    1. Early onset flecked retinal dystrophy associated with new compound heterozygous RPE65 variants in two unrelated Japanese patients. PMID: 29681726
    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. PMID: 27307694
    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. PMID: 25495949
    4. RPE65 variants are the most prevalent causes of Leber congenital amaurosis in Denmark. PMID: 26626312
    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. PMID: 26906952
    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. PMID: 26392743
    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. PMID: 25972377
    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. PMID: 25752820
    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. PMID: 26427430
    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. PMID: 26427455
    11. Expressions of MDSC, FOXP3+TILs, and CTLA-4 are relative stable after nCRT PMID: 26364624
    12. We showed that miR-410 directly regulates predicted target genes OTX2 and RPE65. PMID: 25351180
    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. PMID: 25286304
    14. All RPE65-mutant observers have consistent and substantial losses in temporal acuity and sensitivity compared with normal observers. PMID: 25257057
    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. PMID: 25112876
    16. We identified a novel LCA-related homozygous RPE65 mutation associated with a severe clinical presentation including an early and severe cone dysfunction. PMID: 24771178
    17. properties of disease causing RPE65 with regard to molecular pathogenic mechanism PMID: 24849605
    18. These results strongly suggest that causal mutations in RPE65 are responsible for retinal dystrophy in the affected individuals of consanguineous Pakistani families. PMID: 23878505
    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 . PMID: 23335596
    20. the RPE65-LCA patients had higher variability in kinetic field extent. VA variability in RPE65-LCA fell within reported results for retinitis pigmentosa. PMID: 23341016
    21. Compound heterozygous missense mutations in the RPE65 gene, Leu67Arg and Tyr368Cys, are related to a relatively mild Leber congenital amaurosis phenotype in Chinese patients. PMID: 22509104
    22. Gene therapy for Leber congenital amaurosis caused by RPE65 mutations is sufficiently safe and substantially efficacious in the extrafoveal retina. PMID: 21911650
    23. These data suggest that cone RPE65 supports human diurnal vision, potentially enhancing our strategies for treating Leber congenital amaurosis Type 2. PMID: 22171060
    24. Dominant mutation in RPE65 identified by whole-exome sequencing causes retinitis pigmentosa with choroidal involvement. PMID: 21654732
    25. The structural features of the retina and retinal pigment epithelium in postmortem donor eyes of a 56-year-old patient with a homozygous missense RPE65 mutation correlate the pathology with the patient's visual function. PMID: 21931134
    26. found that the aromatic lipophilic spin traps such as N-tert-butyl-alpha-phenylnitrone (PBN), 2,2-dimethyl-4-phenyl-2H-imidazole-1-oxide (DMPIO), and nitrosobenzene (NB) strongly inhibit RPE65 isomerohydrolase activity in vitro PMID: 21736383
    27. This is the first reported association between compound heterozygous RPE65 mutations and fundus albipunctatus, indicative of a mutation-specific phenotypic effect in this gene. PMID: 21211845
    28. To describe in detail the features of Severe Early Childhood Onset Retinal Dystrophy (SECORD) and differentiate it from Lebers congenital amaurosis, caused by RPE65 mutation. PMID: 20811047
    29. Congenital loss of chromophore production due to RPE65-deficiency together with progressive photoreceptor degeneration cause severe and progressive loss of vision. PMID: 20399883
    30. oxidative stress during the visual cycle results in cleavage of RPE65 PMID: 20510285
    31. FATP1 inhibits 11-cis retinol formation via interaction with the visual cycle retinoid isomerase RPE65 and lecithin:retinol acyltransferase PMID: 20356843
    32. Variations of macular microstructures were observed among LCA (Leber congenital amaurosis) patients with different genotypes. PMID: 19959640
    33. Loss of charge at the E417Q position of RPE65 may represent a mechanism by which the E417Q mutation causes blindness in Leber congenital amaurosis patients. PMID: 20043869
    34. Studies demonstrated improvements in rod and cone visual function in patients with RPE65-LCA administered rAAV2-CBSB-hRPE65. PMID: 19806502
    35. RPE65 is not inherently 11-cis-specific and can produce both 11- and 13-cis isomers, supporting a carbocation (or radical cation) mechanism for isomerization. PMID: 19920137
    36. retinal dystrophy due to paternal isodisomy for chromosome 1 or chromosome 2, with homoallelism for mutations in RPE65 or MERTK, respectively PMID: 11727200
    37. RPE65 mutations present in compound heterozygous form cause severe visual compromise. PMID: 11786058
    38. multiplex PCR follwed by sequencing to screen for mutations in the 14 exons of the RPE65 gene in early-childhood-onset autosomal recessive retinitis pigmentosa and Leber's congenital amaurosis patients PMID: 12357075
    39. The RPE65 mutations K303X and Y431C in compound heterozygous form cause progressive visual compromise that starts in childhood and advances to severe visual loss by the fourth decade of life. PMID: 14962443
    40. Gene therapy with this protein to cure Leber congenital amaurosis; Gene therapy in Rpe65(-/-) mice at advanced-disease stages show some success PMID: 15837919
    41. conserved glutamic acid and histidine residues are essential for the isomerohydrolase activity of RPE65 and its stability PMID: 16198348
    42. AIPL1, CRB1, GUCY2D, RPE65, and RPGRIP1 mutations may have roles in juvenile retinitis pigmentosa PMID: 16272259
    43. We identified and characterised an endemic form of early onset rod-cone dystrophy in a consanguineous population from northeastern Tunisia, due to the prevalence of a single RPE65 mutation. PMID: 16518657
    44. mutations may result in critical structural alterations of RPE65 protein, disrupt its membrane association, and consequently impair its isomerohydrolase activity, leading to retinal degeneration PMID: 16754667
    45. The results demand critical consideration of the human disease mechanism and the therapeutic approach in patients with mutations in the putative visual cycle gene RDH12. PMID: 17197551
    46. Testing confirms the diagnosis at the molecular level and allows for a more precise prognosis of the possible future clinical evolution PMID: 17651254
    47. RPE65 gene mutations represented a significant cause of LCA in the Italian population, whereas GUCY2D and CEP290 mutations had a lower frequency than that found in other reports. PMID: 17724218
    48. Early cone photoreceptor losses in RPE65-LCA suggest that robust RPE65-based visual chromophore production is important for cones. PMID: 17848510
    49. RPE65 from the cone-dominant chicken RPE possesses significantly higher specific retinol isomerohydrolase activity, when compared with RPE65 from rod-dominant species PMID: 18216020
    50. Mutations in the RPE65 gene are rare in patients with leber congenital amaurosis PMID: 18484312

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  • 相關疾?。?/div>
    Leber congenital amaurosis 2 (LCA2); Retinitis pigmentosa 20 (RP20)
  • 亞細胞定位:
    Cytoplasm. Cell membrane; Lipid-anchor. Microsome membrane.
  • 蛋白家族:
    Carotenoid oxygenase family
  • 組織特異性:
    Retina (at protein level). Retinal pigment epithelium specific.
  • 數據庫鏈接:

    HGNC: 10294

    OMIM: 180069

    KEGG: hsa:6121

    STRING: 9606.ENSP00000262340

    UniGene: Hs.2133



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