The DOI matches its Crossref record; 1 of the 1 references it cites were found in a registry or an index.
In vivo dynamics of retinal injury and repair in the rhodopsin mutant dog model of human retinitis pigmentosa
Open accessAbstract
Genetic and environmental factors modify the severity of human neurodegenerations. Retinal degenerations caused by rhodopsin gene mutations show severity differences within and between families and even within regions of the same eye. Environmental light is thought to contribute to this variation. In the naturally occurring dog model of the human disorder, we found that modest light levels, as used in routine clinical practice, dramatically accelerated the neurodegeneration. Dynamics of acute retinal injury (consisting of abnormal intraretinal light scattering) were visualized in vivo in real time with high-resolution optical imaging. Long term consequences included fast or slow retinal degeneration or repair of injury depending on the dose of light exposure. These experiments provide a platform to study mechanisms of neuronal injury, repair, compensation, and degeneration. The data also argue for a gene-specific clinical trial of light reduction in human rhodopsin disease.
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Cites
AP-1 as a regulator of cell life and death
Shaulian, E., & Karin, M. (2002) · Nature Cell Biology · doi:10.1038/ncb0502-e131 · Cited by 2,961
Cited by
Hartong, D. T., Berson, E. L., & Dryja, T. P. (2006) · The Lancet · doi:10.1016/s0140-6736(06)69740-7 · Cited by 2,924
G Protein–Coupled Receptor Rhodopsin
Palczewski, K. (2006) · Annual Review of Biochemistry · doi:10.1146/annurev.biochem.75.103004.142743 · Cited by 764
High-Resolution Imaging with Adaptive Optics in Patients with Inherited Retinal Degeneration
Duncan, J. L., Zhang, Y., Gandhi, J., Nakanishi, C., Othman, M., Branham, K. E. H., Swaroop, A., & Roorda, A. (2007) · Investigative Opthalmology & Visual Science · doi:10.1167/iovs.06-1422 · Cited by 262
Mutation-independent rhodopsin gene therapy by knockdown and replacement with a single AAV vector
Cideciyan, A. V., Sudharsan, R., Dufour, V. L., Massengill, M. T., Iwabe, S., Swider, M., Lisi, B., Sumaroka, A., Marinho, L. F., Appelbaum, T., et al. (2018) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.1805055115 · Cited by 147
Self-citationRetinal Laminar Architecture in Human Retinitis Pigmentosa Caused by Rhodopsin Gene Mutations
Aleman, T. S., Cideciyan, A. V., Sumaroka, A., Windsor, E. A. M., Herrera, W., White, D. A., Kaushal, S., Naidu, A., Roman, A. J., Schwartz, S. B., et al. (2008) · Investigative Opthalmology & Visual Science · doi:10.1167/iovs.07-1110 · Cited by 133
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Metadata
- Authors
- Cideciyan, A. V., Jacobson, S. G., Aleman, T. S., Gu, D., Pearce-Kelling, S. E., Sumaroka, A., Acland, G. M., & Aguirre, G. D.
- Venue
- Proceedings of the National Academy of Sciences
- Published
- 2005-03-22
- Volume
- 102
- Issue
- 14
- Pages
- 5233-5238
- ISSN
- 0027-8424 (ISSN-L)
- 1091-6490
- OpenAlex
- W2101255916
- Cited by
- 187
- References
- 1
- FWCI
- 5.19
- Type
- journal article
- Language
- en
- Open access
- green
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- Cideciyan, A. V., Jacobson, S. G., Aleman, T. S., Gu, D., Pearce-Kelling, S. E., Sumaroka, A., Acland, G. M., & Aguirre, G. D. (2005). In vivo dynamics of retinal injury and repair in the rhodopsin mutant dog model of human retinitis pigmentosa. Proceedings of the National Academy of Sciences. https://doi.org/10.1073/pnas.0408892102
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