Overview

  • Product name

  • Description

    Rabbit polyclonal to RPE65
  • Host species

    Rabbit
  • Tested applications

    Suitable for: WB, ICC/IFmore details
  • Species reactivity

    Reacts with: Rat, Human
    Predicted to work with: Cow, Dog, African green monkey
  • Immunogen

    Recombinant fragment corresponding to Human RPE65 aa 348-484.
    Sequence:

    ENWEEVKKNARKAPQPEVRRYVLPLNIDKADTGKNLVTLPNTTATAILCS DETIWLEPEVLFSGPRQAFEFPQINYQKYCGKPYTYAYGLGLNHFVPDRL CKLNVKTKETWVWQEPDSYPSEPIFVSHPDALEEDDG


    Database link: Q16518

  • Positive control

    • WB: Rat brain tissue lysate. ICC/IF: HeLa cells.

Properties

Applications

Our Abpromise guarantee covers the use of ab235950 in the following tested applications.

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

Application Abreviews Notes
WB 1/500 - 1/5000.
ICC/IF 1/50 - 1/200.

Target

  • Function

    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.
  • Tissue specificity

    Retinal pigment epithelium specific.
  • Involvement in disease

    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.
  • Sequence similarities

    Belongs to the carotenoid oxygenase family.
  • Post-translational
    modifications

    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).
  • Cellular localization

    Cytoplasm. Cell membrane. Attached to the membrane by a lipid anchor when palmitoylated (membrane form), soluble when unpalmitoylated.
  • Information by UniProt
  • Database links

  • Alternative names

    • 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

Images

  • 4% formaldehyde-fixed, 0.2% Triton X-100 permeabilized HeLa (human epithelial cell line from cervix adenocarcinoma) cells labeling RPE65 (green) using ab235950 at 1/233 dilution in ICC/IF. Counterstained with DAPI (blue).

    Cells were blocked in 10% normal Goat Serum. The cells were then incubated with the antibody overnight at 4°C. The secondary antibody was Alexa Fluor® 488-conjugated Goat Anti-Rabbit IgG (H+L).

  • Anti-RPE65 antibody (ab235950) at 1/500 dilution + Rat brain tissue lysate

    Secondary
    Goat polyclonal to rabbit IgG at 1/50000 dilution

References

ab235950 has not yet been referenced specifically in any publications.

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