概述

  • 产品名称Anti-RPE65抗体
    参阅全部 RPE65 一抗
  • 描述
    兔多克隆抗体to RPE65
  • 特异性This antibody do not cross react with other retinal proteins.
  • 经测试应用适用于: WBmore details
  • 种属反应性
    与反应: Mouse
    预测可用于: Rat, Chicken, Cow, Dog, Human, Xenopus laevis, Monkey, Zebrafish
  • 免疫原

    Synthetic peptide corresponding to Mouse RPE65 aa 127-146.
    Sequence:

    EVTDNALVNIYPVGEDYYAC

性能

应用

Our Abpromise guarantee covers the use of ab74998 in the following tested applications.

The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.

应用 Ab评论 说明
WB
  • 应用说明WB: >1/500. Detects a band of approximately 65 kDa (predicted molecular weight: 61 kDa).


    Not yet tested in other applications.
    Optimal dilutions/concentrations should be determined by the end user.
  • 靶标

    • 功能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.
    • 组织特异性Retinal pigment epithelium specific.
    • 疾病相关Defects in RPE65 are the cause of Leber congenital amaurosis type 2 (LCA2) [MIM:204100]. 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) [MIM:613794]. 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. RP20 inheritance is autosomal dominant.
    • 序列相似性Belongs to the carotenoid oxygenase family.
    • 翻译后修饰Palmitoylation by LRAT regulates ligand binding specificity; the palmitoylated form (membrane form) specifically binds all-trans-retinyl-palmitate, while the soluble unpalmitoylated form binds all-trans-retinol (vitamin A).
    • 细胞定位Cytoplasm. Cell membrane. Attached to the membrane by a lipid anchor when palmitoylated (membrane form), soluble when unpalmitoylated.
    • Information by UniProt
    • 数据库链接
    • 别名
      • 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
      see all

    Anti-RPE65 antibody (ab74998)参考文献

    ab74998 has not yet been referenced specifically in any publications.

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