Retinal Pigment Epithelium-Specific Protein 65kDa (RPE65) antibody

Details for Product No. ABIN151819
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Antigen
Synonyms RPE56c, si:ch211-198n5.12, 65kDa, A930029L06Rik, LCA2, Mord1, RP20, rd12, mRPE65, sRPE65
Reactivity
Cow (Bovine), Human, Mouse (Murine), Pig (Porcine)
(22), (21), (18), (16), (14), (12)
Host
Mouse
(19), (10), (6)
Clonality (Clone)
Monoclonal ()
Conjugate
Un-conjugated
(3), (3), (1), (1), (1), (1), (1), (1), (1), (1), (1), (1), (1), (1)
Application
Western Blotting (WB), Flow Cytometry (FACS), Immunocytochemistry (ICC), Immunofluorescence (IF), Immunohistochemistry (IHC), Immunohistochemistry (Frozen Sections) (IHC (fro)), Immunohistochemistry (Paraffin-embedded Sections) (IHC (p))
(25), (10), (8), (8), (5), (4), (3), (2), (2)
Pubmed 10 references available
Quantity 0.1 mL
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Catalog No. ABIN151819
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Immunogen Bovine RPE65 microsomal membrane proteins.
Clone 401-8B11-3D9
Isotype IgG1 kappa
Specificity NB100-355 is specific for RPE65.
Cross-Reactivity (Details) This antibody has only been tested in bovine, porcine, human, mouse,canine. rat,reactivity reported in scientific literature (PMID: 18700924). Chicken reactivity reported in scientific literature (PMID: 24227655)
Purification Protein A purified
Alternative Name RPE65
Background Highly conserved among vertebrate species, RPE65 is a major protein of the retinalpigment epithelium (RPE). RPE65 is essential for the regeneration of rhodopsin in thevisual cycle. Mutations in RPE65 are responsible for certain forms of autosomalrecessive severe retinal dystrophy, including Leber Congenital Amaurosis (LCA). Lossof function results in a block in retinoid processing required for the synthesis of 11-cisretinal and the accumulation of retinyl esters in the RPE. Alternate Names: anti-LCA2 antibody, anti-Leber congenital amaurosis antibody, anti-Retinal pigmentepithelium-specific 65 kDa protein antibody, anti-Retinal pigment epithelium-specificprotein antibody, anti-Retinitis pigmentosa 20 antibody, anti-RP20 antibody.
Gene Symbol: RPE65
Gene ID 6121, 282043
UniProt Q28175
Research Area Signaling, Metabolism, Neurology
Application Notes Immunohistochemistry and western blot. By Western blot, this RPE65 antibody recognizes a band at ~65 kDa, representing bovine RPE65 protein. It has also been tested on recombinant human protein in COS7 cells. It may also be used in immunohistochemistry-Frozen, on murine retinal sections and ICC in porcine.
Recommended dilutions: Flow Cytometry, Immunocytochemistry/Immunofluorescence 1:10-1:500, Immunohistochemistry 1:250, Immunohistochemistry-Frozen 1:250, Immunohistochemistry-Paraffin, Western Blot 1:500 - 1:1000
Protocol Protocol specific for RPE65 Antibody Western Blot
1. Bovine RPE membrane (5ug) and recombinant human RPE65 (~20ug ) expressed in COS7 cells were loaded on a NuPAGE Novex Bis-Tris 10 %gel along with SeeBlue Plus2 marker.
. Run in NuPAGE MOPS electrophoresis buffer (Invitrogen). 3 Samples were probed with NB 100-355 RPE65 antibody (1:5,000 ) followed by alkaline phosphatase-conjugated goat anti-mouse IgG incubation.
. Visualized by NBT/BCIP substrate.
Restrictions For Research Use only
Format Liquid
Concentration 1.0 mg/mL
Buffer PBS, Sodium Azide
Preservative Sodium azide
Precaution of Use WARNING: Reagents contain sodium azide. Sodium azide is very toxic if ingested or inhaled. Avoid contact with skin, eyes, or clothing. Wear eye or face protection when handling. If skin or eye contact occurs, wash with copious amounts of water. If ingested or inhaled, contact a physician immediately. Sodium azide yields toxic hydrazoic acid under acidic conditions. Dilute azide-containing compounds in running water before discarding to avoid accumulation of potentially explosive deposits in lead or copper plumbing.
Handling Advice Avoid freeze-thaw cycles
Storage -20 °C
Storage Comment Aliquot and store at -20 °C or -80 °C.
Supplier Images
anti-Retinal Pigment Epithelium-Specific Protein 65kDa (RPE65) antibody RPE65 detected in bovine and human samples. Lane 1: bovine RPE membrane, lane 2: recombinant human RPE transfected COS7 cell lysate.
anti-Retinal Pigment Epithelium-Specific Protein 65kDa (RPE65) antibody (2) Immunohistochemical staining of RPE65 in mouse retina tissue using ABIN151819.
General Nicoletti, Wong, Kawase et al.: "Molecular characterization of the human gene encoding an abundant 61 kDa protein specific to the retinal pigment epithelium." in: Human molecular genetics, Vol. 4, Issue 4, pp. 641-9, 1995 (PubMed).

Hamel, Tsilou, Pfeffer et al.: "Molecular cloning and expression of RPE65, a novel retinal pigment epithelium-specific microsomal protein that is post-transcriptionally regulated in vitro." in: The Journal of biological chemistry, Vol. 268, Issue 21, pp. 15751-7, 1993 (PubMed).

Redmond, Yu, Lee et al.: "Rpe65 is necessary for production of 11-cis-vitamin A in the retinal visual cycle." in: Nature genetics, Vol. 20, Issue 4, pp. 344-51, 1998 (PubMed).

Thompson, Gyuerues, Fleischer et al.: "Genetics and phenotypes of RPE65 mutations in inherited retinal degeneration." in: Investigative ophthalmology & visual science, Vol. 41, Issue 13, pp. 4293-9, 2000 (PubMed).

Jacobson, Yan, Gao et al.: "Aging enhances pressure-induced arterial superoxide formation." in: American journal of physiology. Heart and circulatory physiology, Vol. 293, Issue 3, pp. H1344-50, 2007 (PubMed).

Jacobson, Aleman, Cideciyan et al.: "Human cone photoreceptor dependence on RPE65 isomerase." in: Proceedings of the National Academy of Sciences of the United States of America, Vol. 104, Issue 38, pp. 15123-8, 2007 (PubMed).

Jiang, Ke, Sun et al.: "Reactivation of uveitogenic T cells by retinal astrocytes derived from experimental autoimmune uveitis-prone B10RIII mice." in: Investigative ophthalmology & visual science, Vol. 49, Issue 1, pp. 282-9, 2008 (PubMed).

Lall, Ferrell, Nagar et al.: "Iron regulates L-cystine uptake and glutathione levels in lens epithelial and retinal pigment epithelial cells by its effect on cytosolic aconitase." in: Investigative ophthalmology & visual science, Vol. 49, Issue 1, pp. 310-9, 2008 (PubMed).

Chen, Muckersie, Robertson et al.: "Characterization of a spontaneous mouse retinal pigment epithelial cell line B6-RPE07." in: Investigative ophthalmology & visual science, Vol. 49, Issue 8, pp. 3699-706, 2008 (PubMed).

Harris, Fisher, Jorgensen et al.: "CD133 progenitor cells from the bone marrow contribute to retinal pigment epithelium repair." in: Stem cells (Dayton, Ohio), Vol. 27, Issue 2, pp. 457-66, 2009 (PubMed).

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