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RPE65

retinal pigment epithelium-specific protein 65kDa

Plays important roles in the production of 11-cis retinal and in visual pigment regeneration. The soluble form binds vitamin A (all-trans-retinol), making it available for LRAT processing to all-trans-retinyl ester. The membrane form, palmitoylated by LRAT, binds all-trans-retinyl esters, making them available for IMH (isomerohydrolase) processing to all-cis-retinol. The soluble form is regenerated by transferring its palmitoyl groups onto 11-cis-retinol, a reaction catalyzed by LRAT. The enzymatic activity is linearly dependent of the expression levels and membrane association.

Gene Name:	retinal pigment epithelium-specific protein 65kDa
Synonyms:	RPE65, LCA2, RBP-binding membrane protein, Retinol isomerase, SRPE65, Rd12, MRPE65, Retinoid isomerohydrolase, RP20
Target Sequences:	NM_000329 NP_000320.1 Q16518

Publications (18)

Aging enhances pressure-induced arterial superoxide formation. Jacobson A, Yan C, Gao Q, Rincon-Skinner T, Rivera A, Edwards J, Huang A, Kaley G, Sun D. American journal of physiology. Heart and circulatory physiology. 2007 293:H1344-50. [PubMed:17557915]

Human cone photoreceptor dependence on RPE65 isomerase. Jacobson SG, Aleman TS, Cideciyan AV, Heon E, Golczak M, Beltran WA, Sumaroka A, Schwartz SB, Roman AJ, Windsor EA, Wilson JM, Aguirre GD, Stone EM, Palczewski K. Proceedings of the National Academy of Sciences of the United States of America. 2007 104:15123-8. (WB; Human) [PubMed:17848510] [PMC:PMC1986623]

Reactivation of uveitogenic T cells by retinal astrocytes derived from experimental autoimmune uveitis-prone B10RIII mice. Jiang G, Ke Y, Sun D, Han G, Kaplan HJ, Shao H. Investigative ophthalmology & visual science. 2008 49:282-9. (ICC; Mouse) [PubMed:18172104] [PMC:PMC2567872]

Iron regulates L-cystine uptake and glutathione levels in lens epithelial and retinal pigment epithelial cells by its effect on cytosolic aconitase. Lall MM, Ferrell J, Nagar S, Fleisher LN, McGahan MC. Investigative ophthalmology & visual science. 2008 49:310-9. (IHC-P; Dog) [PubMed:18172108]

Characterization of a spontaneous mouse retinal pigment epithelial cell line B6-RPE07. Chen M, Muckersie E, Robertson M, Fraczek M, Forrester JV, Xu H. Investigative ophthalmology & visual science. 2008 49:3699-706. (WB; Mouse) [PubMed:18421091]

CD133 progenitor cells from the bone marrow contribute to retinal pigment epithelium repair. Harris JR, Fisher R, Jorgensen M, Kaushal S, Scott EW. Stem cells (Dayton, Ohio). 2009 27:457-66. (IHC-Fr; Mouse) [PubMed:19038791]

Steroids do not prevent photoreceptor degeneration in the light-exposed T4R rhodopsin mutant dog retina irrespective of AP-1 inhibition. Gu D, Beltran WA, Pearce-Kelling S, Li Z, Acland GM, Aguirre GD. Investigative ophthalmology & visual science. 2009 50:3482-94. (IHC-Fr; Dog) [PubMed:19234347] [PMC:PMC2742955]

Retinal Astrocytes respond to IL-17 differently than Retinal Pigment Epithelial cells. Ke Y, Jiang G, Sun D, Kaplan HJ, Shao H. Journal of leukocyte biology. 2009 86:1377-84. (ICC; Mouse) [PubMed:19690291] [PMC:PMC2780918]

Equivalence of conventionally-derived and parthenote-derived human embryonic stem cells. Harness JV, Turovets NA, Seiler MJ, Nistor G, Altun G, Agapova LS, Ferguson D, Laurent LC, Loring JF, Keirstead HS. PloS one. 2011 6:e14499. (ICC, WB; Human) [PubMed:21249129] [PMC:PMC3017547]

ZIP2 and ZIP4 mediate age-related zinc fluxes across the retinal pigment epithelium. Leung KW, Gvritishvili A, Liu Y, Tombran-Tink J. Journal of molecular neuroscience : MN. 2012 46:122-37. (ICC, IHC, Flo; Mouse, Human) [PubMed:21603979]

Short-interfering RNAs induce retinal degeneration via TLR3 and IRF3. Kleinman ME, Kaneko H, Cho WG, Dridi S, Fowler BJ, Blandford AD, Albuquerque RJ, Hirano Y, Terasaki H, Kondo M, Fujita T, Ambati BK, Tarallo V, Gelfand BD, Bogdanovich S, Baffi JZ, Ambati J. Molecular therapy : the journal of the American Society of Gene Therapy. 2012 20:101-8. (IHC; Mouse) [PubMed:21988875] [PMC:PMC3255577]

Enhanced retinal pigment epithelium regeneration after injury in MRL/MpJ mice. Xia H, Krebs MP, Kaushal S, Scott EW. Experimental eye research. 2011 93:862-72. (IHC-Fr; Mouse) [PubMed:21989111] [PMC:PMC3249660]

Generation of retinal pigment epithelial cells from small molecules and OCT4 reprogrammed human induced pluripotent stem cells. Ukrohne TU, Westenskow PD, Kurihara T, Friedlander DF, Lehmann M, Dorsey AL, Li W, Zhu S, Schultz A, Wang J, Siuzdak G, Ding S, Friedlander M. Stem cells translational medicine. 2012 1:96-109. [PubMed:22532929] [PMC:PMC3328503]

Generation of retinal pigment epithelial cells from human embryonic stem cell-derived spherical neural masses. Cho MS, Kim SJ, Ku SY, Park JH, Lee H, Yoo DH, Park UC, Song SA, Choi YM, Yu HG. Stem cell research. 2012 9:101-9. (ICC; Human) [PubMed:22683799]

Retinal astrocytes pretreated with NOD2 and TLR2 ligands activate uveitogenic T cells. Jiang G, Sun D, Kaplan HJ, Shao H. PloS one. 2012 7:e40510. (ICC; Mouse) [PubMed:22808176] [PMC:PMC3393697]

Histology and immunochemistry evaluation of autologous translocation of retinal pigment epithelium-choroid graft in porcine eyes. Fernandez-Bueno I, Rodriguez de la Rua E, Hileeto D, Parrado ML, Regueiro-Purrios M, Sala-Puigdollers A, Srivastava GK, Gonzalo-Orden JM, Pastor JC. Acta ophthalmologica. 2013 91:e125-32. (ICC, IHC; Pig) [PubMed:23256869]

Regulation of T-lymphocyte CCL3 and CCL4 production by retinal pigment epithelial cells. Wallace CA, Moir G, Malone DF, Duncan L, Devarajan G, Crane IJ. Investigative ophthalmology & visual science. 2013 54:722-30. [PubMed:23299481]

Endogenous regeneration of damaged retinal pigment epithelium following low dose sodium iodate administration: an insight into the role of glial cells in retinal repair. Machali[Character 144]ska A, Kawa MP, Pius-Sadowska E, Rogi[Character 144]ska D, K[Character 142]os P, Baumert B, Wiszniewska B, Machali[Character 144]ski B. Experimental eye research. 2013 Jul;112:68-78. [Full Text Article] [PubMed:23623997] Related Antibodies: LS-B90.

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For RESEARCH USE ONLY. Intended for use by laboratory professionals. Not intended for human diagnostic or therapeutic purposes.

The data on this page has been compiled from LifeSpan internal sources, the National Center for Biotechnology Information (NCBI), and The Universal Protein Resource (UniProt).

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