Retinal Pigment Epithelium Specific Protein (Retinal Pigment Epithelium-specific 65kD Protein, RPE65, All-trans-retinyl-palmitate Hydrolase, LCA2, mRPE65, rd12, Retinol Isomerase, Retinoid Isomerohydrolase, RP20, sRPE65) (HRP)

Highly conserved among vertebrate species, RPE65 is a major protein of the retinal pigment epithelium (RPE). RPE65 is essential for the regeneration of rhodopsin in the visual cycle. Mutations in RPE65 are responsible for certain forms of autosomal recessive severe retinal dystrophy, including Leber Congenital Amaurosis (LCA). Loss of function results in a block in retinoid processing required for the synthesis of 11-cis retinal and the accumulation of retinyl esters in the RPE.\n\nApplications:\nSuitable for use in Western Blot, Immunohistochemistry and Immunocyctochemistry. Other applications not tested.\n\nRecommended Dilutions:\nWestern Blot: 1:5000-1:10,000, recognizes a band at 65kD, representing bovine RPE65 protein.\nImmunohistochemistry: 1:250\nOptimal dilutions to be determined by researcher.\n\nStorage and Stability:\nStore at 2-8 degrees C. Do Not Freeze! Aliquots are stable for at least 3 months. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap. Further dilutions can be made in assay buffer. Sodium azide is a potent inhibitor of peroxidase and should not be added to HRP conjugates.

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SPECIFICATIONS

Catalog Number

R1659-70D

Size

100ul

Applications

ICC, IHC, WB

Hosts

Mouse

Reactivities

Bov

Form

Supplied as a liquid in PBS, pH 7.4. Labeled with HRP.

P Type

Mab

Purity

Purified by Protein A affinity chromatography.

Isotype

IgG1,k

References

1. Jacobson, A., C. Yan, et al. (2007) 293:H1344-1350. 2. Jacobson, S. G., T. S. Aleman, et al. (2007) 104:15123-15128. 3. Jiang, G., et al. (2008) Invest. Ophthalmol. Vis. Sci. Jan 49:282-289. 4. Lall, M. M., et al. (2008) Invest. Ophthalmol. Vis. Sci. Jan 49:310-319. 5. Chen M, et al. (2008) Invest Ophthalmol Vis Sci 49(8):3699-3706. 6. Harris JR, et al. (2008) Stem Cells 2008-0836. 7. Nicoletti, A., et al. (1995) Hum. Mol. Genet. 4:641-649. 8. Thompson, D.A., et al. (2000) Invest. Ophthalmol. Vis. Sci. 41:4293-42999. 9. Redmond, T.M. et al. (1998) Nat. Genet. 20:344-351. 10. Hamel, C.P., et al. (1993) JBC. 268(21):15751-15757.

Additional Info

Recognizes RPE65. Tested in bovine, porcine, human and murine.

Alternative Names

Retinal Pigment Epithelium-specific Protein 65kD, EC=3.1.1.64, EC=5.2.1.7, Leber Congenital Amaurosis, Retinitis Pigmentosa 20