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R1659-70D Mouse Anti-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)

Specifications
References
Clone Type
Monoclonal
Host
Mouse
Source
Bovine
Swiss Prot
Q28175
Conjugate
HRP
Isotype
IgG1,k
Clone Number
8B99 (401.8B11.3D9)
Grade
Affinity Purified
Applications
IHC WB
Crossreactivity
Bo Ca Ch Hu Mo Rt Xe
Gene #
RPE65
Shipping Temp
Blue Ice
Storage Temp
-20°C
Notes
Preservative Free
Retinal Pigment Epithelium-specific Protein 65kD, EC=3.1.1.64, EC=5.2.1.7, Leber Congenital Amaurosis, Retinitis Pigmentosa 20

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.

Applications
Suitable for use in Western Blot and Immunohistochemistry. Other applications not tested.
Recommended Dilutions
Immunohistochemistry: paraffin/frozen Optimal dilutions to be determined by researcher.
Storage and Stability
Store product at 4°C if to be used immediately within two weeks. For long-term storage, aliquot to avoid repeated freezing and thawing and store at -20°C. Aliquots are stable at -20°C for 12 months after receipt. Dilute required amount only prior to immediate use. Further dilutions can be made in assay buffer. Note: Sodium azide is a potent inhibitor of peroxidase and should not be added to HRP conjugates. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap.
Immunogen
Bovine RPE microsomal membrane proteins. |Cellular Localization: Cytoplasmic, microsomeal and cell membrane. Species Sequence Homology: birds; 89%, zebrafish; 74%, sea lamprey; 71%
Form
Supplied as a liquid in PBS. No preservative added. Labeled with horseradish peroxidase (HRP)
Purity
Purified by Protein G affinity chromatography.
Specificity
Recognizes bovine RPE65. Species Crossreactivity: porcine, human, mouse, rat, canine, chicken, primate and Xenopus
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.
USBio References
No references available
Conjugates
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