抗RPE65 (401.8B11.3D9)抗体 | Anti-RPE65 (401.8B11.3D9) Antibody

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	Immunohistochemistry-Frozen: RPE65 Antibody (401.8B11.3D9) [NB100-355] - Staining of RPE65 in a cryosection of mouse retina tissue using RPE65 antibody (clone 401.8B11.3D9).
	Western Blot: RPE65 Antibody (401.8B11.3D9) [NB100-355] - WB analysis of RPE65 in 20ug lysate of COS7 cells expressing recombinant Human RPE65 (Lane 1) and 5ug of Bovine retinal pigment epithelium membrane fraction (Lane 2). Blot processed for detection with alkaline phosphatase conjugated goat-anti Mouse IgG secondary antibody and NBT/BCIP substrate.
	Immunocytochemistry/Immunofluorescence: RPE65 Antibody (401.8B11.3D9) [NB100-355] - ICC-IF analysis of cultured ARPE19 cells, a spontaneously arising human retinal pigment epithelia cell line - 10 minutes fixation in 4% PFA, 10 minutes permeabilization in PBS containing 0.2% Triton X-100 (PBS-T), 1 hour blocking in 10% normal goat serum containing 1% BSA in PBS-T, 1:100 primary antibody dilution in PBS, ON 4C incubation.
	Immunohistochemistry-Paraffin: RPE65 Antibody (401.8B11.3D9) [NB100-355] - IHC analysis of formalin fixed paraffin-embedded (FFPE) human glioblastoma using Histone H3 [pS10] antibody at 1:500 on a Bond Rx autostainer (Leica Biosystems). The assay involved 20 minutes of heat induced antigen retrieval (HIER) using 10mM sodium citrate buffer (pH 6.0) and endogenous peroxidase quenching with peroxide block. The sections were incubated with primary antibody for 30 minutes and Bond Polymer Refine Detection (Leica Biosystems) with DAB was used for signal development followed by counterstaining with hematoxylin. Whole slide scanning and capturing of representative images was performed using Aperio AT2 (Leica Biosystems). Cytoplasmic staining of RPE65 was observed. Staining was performed by Histowiz.
	Immunocytochemistry: RPE65 Antibody (401.8B11.3D9) [NB100-355] - ARPE-19 monolayer on glass substrate. Fixative Methanol Blocking Goat Serum. Dilution 1:250. Incubation 1h RT. This image was submitted via customer Review.

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Background

RPE65 (retinal pigment epithelium-specific 65 kDa protein), also called retinoid isomerohydrolase, is an essential retinoid isomerase enzyme of the canonical retinal pigment epithelium/RPE visual cycle and it is highly /preferentially expressed in the RPE layer, monolayer of neuroepithelium-derived cells located between the choroid and photoreceptors of the eye. It is responsible for retinol isomerization (essential reaction required for functional vision) and it converts all-trans retinyl ester to 11-cis retinol. RPE65 protein is functionally conserved throughout the vertebrates, and it is commonly used as an RPE marker by the research community. The loss of RPE65 function has been suggested to involve two distinct mechanisms - loss of catalytic activity and a lower expression level of RPE65. More than 80 different mutations have been reported in this protein and RPE65 gene mutations have been shown to be the cause of Leber's congenital amaurosis (LCA, MIM204000), a severe congenital or early infant-onset form of inherited retinal dystrophy.

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