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

Details for Product No. ABIN314161
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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), (20), (18), (16), (14), (12)
Host
Mouse
(19), (10), (5)
Clonality (Clone)
Monoclonal ()
Conjugate
Biotin
(3), (3), (1), (1), (1), (1), (1), (1), (1), (1), (1), (1), (1), (1)
Application
Western Blotting (WB), Immunocytochemistry (ICC), Immunofluorescence (IF), Immunohistochemistry (IHC), Immunohistochemistry (Frozen Sections) (IHC (fro))
(24), (10), (8), (7), (5), (4), (3), (2), (2)
Pubmed 10 references available
Quantity 0.1 mL
Shipping to United States (Change)
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Catalog No. ABIN314161
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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, 19892, 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: Western Blot 1:5000-1:10000, Immunohistochemistry-Frozen 1:250, Immunocytochemistry/Immunofluorescence 1:10-1:500, Immunohistochemistry 1:250
Restrictions For Research Use only
Format Liquid
Concentration 1.0 mg/mL
Buffer PBS
Preservative Without preservative
Storage 4 °C
Storage Comment 4 °C in the dark
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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