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The Journal of Neuroscience, February 18, 2009, 29(7):2297-2308; doi:10.1523/JNEUROSCI.2034-08.2009

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Neurobiology of Disease
Loss of the Cholesterol-Binding Protein Prominin-1/CD133 Causes Disk Dysmorphogenesis and Photoreceptor Degeneration

Serena Zacchigna,^1,2 * Hideyasu Oh,^1,2 * Michaela Wilsch-Bräuninger,^3 * Ewa Missol-Kolka,^3,4 József Jászai,⁴ Sandra Jansen,^1,2 Naoyuki Tanimoto,⁵ Felix Tonagel,⁵ Mathias Seeliger,⁵ Wieland B. Huttner,³ Denis Corbeil,^3,4 Mieke Dewerchin,^1,2 Stefan Vinckier,^1,2 Lieve Moons,^1,2 and Peter Carmeliet^1,2

¹Vesalius Research Center, Vlaams Instituut voor Biotechnologie, and ²Vesalius Research Center, Katholieke Universiteit Leuven, B-3000 Leuven, Belgium, ³Max Planck Institute of Molecular Cell Biology and Genetics, and ⁴Tissue Engineering Laboratories, Biotec, Technical University, D-01307 Dresden, Germany, and ⁵Ocular Neurodegeneration Research Group, Centre for Ophtalmology, Institute for Opthalmic Research, D-72076 Tübingen, Germany

Correspondence should be addressed to Peter Carmeliet at the above address. Email: peter.carmeliet{at}med.kuleuven.be

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.

Key words: degeneration; retina; blindness; knockout; apoptosis; eye

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Received May 2, 2008; revised Dec. 28, 2008; accepted Jan. 18, 2008.

Correspondence should be addressed to Peter Carmeliet at the above address. Email: peter.carmeliet{at}med.kuleuven.be

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