Abstract
Prominin-1/CD133 (Prom-1) is a commonly used marker of neuronal, vascular, hematopoietic and other stem cells, yet little is known about its biological role and importance in vivo. Here, we show that loss of Prom-1 results in progressive degeneration of mature photoreceptors with complete loss of vision. Despite the expression of Prom-1 on endothelial progenitors, photoreceptor degeneration was not attributable to retinal vessel defects, but caused by intrinsic photoreceptor defects in disk formation, outer segment morphogenesis, and associated with visual pigment sorting and phototransduction abnormalities. These findings shed novel insight on how Prom-1 regulates neural retinal development and phototransduction in vertebrates.
Publication types
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Research Support, Non-U.S. Gov't
MeSH terms
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AC133 Antigen
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Animals
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Antigens, CD / genetics*
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Cholesterol / metabolism
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Down-Regulation / genetics
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Endothelial Cells / cytology
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Endothelial Cells / metabolism
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Genetic Predisposition to Disease / genetics
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Glycoproteins / genetics*
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Mice
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Mice, Inbred C57BL
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Mice, Knockout
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Nerve Degeneration / genetics
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Nerve Degeneration / metabolism
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Nerve Degeneration / physiopathology
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Peptides / genetics*
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Photoreceptor Cells, Vertebrate / metabolism*
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Photoreceptor Cells, Vertebrate / pathology
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Retina / abnormalities*
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Retina / metabolism*
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Retina / physiopathology
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Retinal Artery / abnormalities
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Retinal Artery / physiopathology
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Retinal Degeneration / genetics*
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Retinal Degeneration / metabolism*
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Retinal Degeneration / physiopathology
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Retinal Pigments / metabolism
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Stem Cells / cytology
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Stem Cells / metabolism
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Vision, Ocular / genetics
Substances
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AC133 Antigen
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Antigens, CD
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Glycoproteins
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Peptides
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Prom1 protein, mouse
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Retinal Pigments
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Cholesterol