Abstract
Retinaldehyde adducts (bisretinoids) accumulate in retinal pigment epithelial (RPE) cells as lipofuscin. Bisretinoids are implicated in some inherited and age-related forms of macular degeneration that lead to the death of RPE cells and diminished vision. By comparing albino and black-eyed mice and by rearing mice in darkness and in cyclic light, evidence indicates that bisretinoid fluorophores undergo photodegradation in the eye (Ueda et al. Proc Natl Acad Sci 113:6904-6909, 2016). Given that the photodegradation products modify and impair cellular and extracellular molecules, these processes likely impart cumulative damage to retina.
Keywords:
ABCA4-associated disease; Age-related macular degeneration; Bisretinoid; Lipofuscin; Retinal degeneration; Retinaldehyde; Visual cycle; Vitamin E.
MeSH terms
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ATP-Binding Cassette Transporters / deficiency
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Albinism / metabolism
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Albinism / pathology
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Amines / metabolism
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Animals
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Antioxidants / pharmacology
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Antioxidants / therapeutic use
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Darkness
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Eye Color*
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Free Radical Scavengers / pharmacology
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Free Radical Scavengers / therapeutic use
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Light
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Lipofuscin / metabolism
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Lipofuscin / radiation effects*
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Macular Degeneration / congenital
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Macular Degeneration / etiology
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Macular Degeneration / genetics
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Macular Degeneration / metabolism*
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Macular Degeneration / prevention & control
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Melanosis / metabolism
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Melanosis / pathology
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Mice
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Mice, Inbred C57BL
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Mice, Mutant Strains
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Photochemistry
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Retinal Pigment Epithelium / metabolism*
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Retinaldehyde / metabolism
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Rod Cell Outer Segment / metabolism
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Stargardt Disease
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Vitamin E / pharmacology
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Vitamin E / therapeutic use
Substances
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ATP-Binding Cassette Transporters
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Abca4 protein, mouse
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Amines
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Antioxidants
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Free Radical Scavengers
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Lipofuscin
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Vitamin E
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Retinaldehyde