期刊
JOURNAL OF NEUROSCIENCE
卷 29, 期 7, 页码 2297-2308出版社
SOC NEUROSCIENCE
DOI: 10.1523/JNEUROSCI.2034-08.2009
关键词
degeneration; retina; blindness; knockout; apoptosis; eye
资金
- Bristol-Myers-Squibb, Methusalem Funding
- GOA
- German Research Council [ Deutsche Forschungsgemeinschaft (DFG) [Se837/4-1, 5-1, 6-1]
- European Union [LSHG-CT-512036]
- DFG [SPP 1109, Hu275/7-3, CO 298/2-2, SPP 1111, Hu275/8-3, SFB/TR13-04, B1, SFB 655, A2, A13]
- European Union Seventh Framework Program via a Marie Curie Intra European fellowship
- Japan Society for the Promotion of Science
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.
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