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

Antigen: Retinal Pigment Epithelium-Specific Protein 65kDa (RPE65)

Synonyms: LCA2, RP20, rd12, mRPE65, sRPE65, LOC100004076

Clonality: Monoclonal (401-8B11-3D9)

Host: Mouse

Reactivity: Cow (Bovine)

Conjugate: Un-conjugated

Application: Immunohistochemistry (Frozen Sections) (IHC (fro)), Immunofluorescence (IF), Western Blotting (WB)

Catalog no.: ABIN314161

Additional Information

Alternative name: RPE65

Gene ID: 6121, 19892, 282043

UniProt: Q28175

Immunogen: Bovine RPE microsomal membrane proteins.

Cross-Reactivity: Cow (Bovine), Human, Mouse (Murine), Pig (Porcine)

Isotype: IgG1

Clone: 401-8B11-3D9

Description: 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.

Specificity: ABIN151819 is specific for RPE65. Species Reactivity: This antibody has only been tested in bovine, porcine, human and murine. Please note the applications. Other species have not been tested. Localization: Cytoplasmic.

Application Details

Application Notes: Suggested working dilutions: immunohistochemistry 1:250,Western Blot 1:5000-1:10000,Immunocytochemistry

Concentration: 1.0 mg/ml

Purification: Purified

Buffer: PBS. Preservative: 0.1% Sodium Azide.

Storage: Aliquot and store at -20C or -80C. Avoid freeze-thaw cycles.

Research Area: Signaling, Metabolism, Neurology

Restrictions: For Research Use only

Publications

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).