Membrane-binding and enzymatic properties of RPE65
Open accessAbstract
Regeneration of visual pigments is essential for sustained visual function. Although the requirement for non-photochemical regeneration of the visual chromophore, 11-cis-retinal, was recognized early on, it was only recently that the trans to cis retinoid isomerase activity required for this process was assigned to a specific protein, a microsomal membrane enzyme called RPE65. In this review, we outline progress that has been made in the functional characterization of RPE65. We then discuss general concepts related to protein-membrane interactions and the mechanism of the retinoid isomerization reaction and describe some of the important biochemical and structural features of RPE65 with respect to its membrane-binding and enzymatic properties.
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11-cis-retinal is produced in the RPE and delivered to photoreceptors.
“The photosensitive active retinoid, 11-cis-retinal, is produced in the RPE and delivered to the photoreceptors.”
From Chemistry of the Retinoid (Visual) Cycle (2014) · cites this paper as [284]
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[21] Multienzyme analysis of visual cycle
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Self-citationExtraction of Membrane Proteins
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Negative charge of the glutamic acid 417 residue is crucial for isomerohydrolase activity of RPE65
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“Mutagenesis experiments combined with mutations observed in LCA patients have established that the second shell Glu residues are essential for RPE65 isomerase activity and even highly similar residues such as Asp and Gln are unable to functionally substitute for them (Hanein et al., 2004; Nikolaeva et al., 2010; Redmo…”
Monoclonal antibodies which recognize endoplasmic reticulum in the retinal pigment epithelium
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CRALBP Ligand and Protein Interactions
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Chemistry of the Retinoid (Visual) Cycle
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Self-citationKey enzymes of the retinoid (visual) cycle in vertebrate retina
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Self-citationSpatial Distribution of the Pathways of Cholesterol Homeostasis in Human Retina
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Self-citationEvolution of Mammalian Opn5 as a Specialized UV-absorbing Pigment by a Single Amino Acid Mutation
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Systems pharmacology identifies drug targets for Stargardt disease–associated retinal degeneration
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Self-citationRetinyl ester hydrolases and their roles in vitamin A homeostasis
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Structural and mechanistic aspects of carotenoid cleavage dioxygenases (CCDs)
Daruwalla, A., & Kiser, P. D. (2019) · Biochimica et Biophysica Acta (BBA) - Molecular and Cell Biology of Lipids · doi:10.1016/j.bbalip.2019.158590 · Cited by 70 · see citing works
Self-citationChemistry and Biology of the Initial Steps in Vision: The Friedenwald Lecture
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Self-citationKiser, P. D., Farquhar, E. R., Shi, W., Sui, X., Chance, M. R., & Palczewski, K. (2012) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.1212025109 · Cited by 63 · see citing works
Self-citationUtilization of Dioxygen by Carotenoid Cleavage Oxygenases
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Survival of an HLA-mismatched, bioengineered RPE implant in dry age-related macular degeneration
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Gene therapy provides long-term visual function in a pre-clinical model of retinitis pigmentosa
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Pathways and disease-causing alterations in visual chromophore production for vertebrate vision
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Inflammatory priming predisposes mice to age-related retinal degeneration
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Self-citationRPE65-related retinal dystrophy: Mutational and phenotypic spectrum in 45 affected patients
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Self-citationEidet, J. R., Reppe, S., Pasovic, L., Olstad, O. K., Lyberg, T., Khan, A. Z., Fostad, I. G., Chen, D. F., & Utheim, T. P. (2016) · Scientific Reports · doi:10.1038/srep22671 · Cited by 26 · see citing works
Vitamin A and its natural derivatives
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Retinol dehydrogenases: Membrane-bound enzymes for the visual function
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Retinal glial remodeling by FGF21 preserves retinal function during photoreceptor degeneration
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Human Cellular Retinaldehyde-Binding Protein Has Secondary Thermal 9-cis-Retinal Isomerase Activity
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Self-citationStructural approaches to understanding retinal proteins needed for vision
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Complementation Test of Rpe65 Knockout and Tvrm148
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An inducible amphipathic α-helix mediates subcellular targeting and membrane binding of RPE65
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- Authors
- Kiser, P. D., & Palczewski, K.
- Affiliations
- Case Western Reserve University (US)
- Corresponding author
- Palczewski, Krzysztof
- Venue
- Progress in Retinal and Eye Research
- Publisher
- Elsevier BV
- Published
- 2010-09
- Volume
- 29
- Issue
- 5
- Pages
- 428-442
- ISSN
- 1350-9462 (ISSN-L)
- PMC
- PMC2903629
- OpenAlex
- W2118546994
- Semantic Scholar
- cc2f86edd7afdbfa6320ac2f30ea97dbf4fcc512
- Cited by
- 62
- References
- 120
- Citations by year
- 2026: 1 · 2025: 1 · 2024: 2 · 2023: 2 · 2022: 3 · 2021: 3 · 2020: 4 · 2019: 4 · 2018: 2 · 2017: 5 · 2016: 3 · 2015: 5 · 2014: 8 · 2013: 6 · 2012: 7
- FWCI
- 1.07
- Type
- journal article
- Language
- en
- Open access
- green
- www.ncbi.nlm.nih.gov
- Locations
- Progress in Retinal and Eye Research (publishedVersion)
- PubMed (publishedVersion)
- Europe PMC (PubMed Central) (acceptedVersion)
- PubMed Central (acceptedVersion)
- Funders
- National Eye Institute (T32EY007157, R01EY009339)
- Topics
- Life Sciences › Biochemistry, Genetics and Molecular Biology › Molecular Biology › Retinal Development and Disorders
- Life Sciences › Neuroscience › Cellular and Molecular Neuroscience › Photoreceptor and optogenetics research
- Life Sciences › Biochemistry, Genetics and Molecular Biology › Molecular Biology › Retinoids in leukemia and cellular processes
- Keywords
- RPE65, 11-cis-retinal, visual pigment regeneration, protein-membrane interactions, membrane binding
- MeSH terms
- Retinoid Isomerohydrolase, Animals, Carrier Proteins, Eye Proteins, Humans, Protein Binding, cis-trans-Isomerases, Retinal Pigment Epithelium, Leber Congenital Amaurosis
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