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A retinitis pigmentosa GTPase regulator (RPGR)- deficient mouse model for X-linked retinitis pigmentosa (RP3)
Open accessAbstract
The X-linked RP3 locus codes for retinitis pigmentosa GTPase regulator (RPGR), a protein of unknown function with sequence homology to the guanine nucleotide exchange factor for Ran GTPase. We created an RPGR-deficient murine model by gene knockout. In the mutant mice, cone photoreceptors exhibit ectopic localization of cone opsins in the cell body and synapses and rod photoreceptors have a reduced level of rhodopsin. Subsequently, both cone and rod photoreceptors degenerate. RPGR was found normally localized to the connecting cilia of rod and cone photoreceptors. These data point to a role for RPGR in maintaining the polarized protein distribution across the connecting cilium by facilitating directional transport or restricting redistribution. The function of RPGR is essential for the long-term maintenance of photoreceptor viability.
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Cited by
The Ciliopathies: An Emerging Class of Human Genetic Disorders
Badano, J. L., Mitsuma, N., Beales, P. L., & Katsanis, N. (2006) · Annual Review of Genomics and Human Genetics · doi:10.1146/annurev.genom.7.080505.115610 · Cited by 1,144
Chang, B., Khanna, H., Hawes, N., Jimeno, D., He, S., Lillo, C., Parapuram, S. K., Cheng, H., Scott, A., Hurd, R. E., et al. (2006) · Human Molecular Genetics · doi:10.1093/hmg/ddl107 · Cited by 328
Beltran, W. A., Cideciyan, A. V., Lewin, A. S., Iwabe, S., Khanna, H., Sumaroka, A., Chiodo, V. A., Fajardo, D. S., Román, A. J., Deng, W.-T., et al. (2012) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.1118847109 · Cited by 260
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
The role of primary cilia in the development and disease of the retina
Wheway, G., Parry, D. A., & Johnson, C. A. (2014) · Organogenesis · doi:10.4161/org.26710 · Cited by 152
Metadata
- Authors
- Hong, D.-H., Pawlyk, B. S., Shang, J., Sandberg, M. A., Berson, E. L., & Li, T.
- Venue
- Proceedings of the National Academy of Sciences
- Published
- 2000-03-21
- Volume
- 97
- Issue
- 7
- Pages
- 3649-3654
- ISSN
- 0027-8424 (ISSN-L)
- 1091-6490
- OpenAlex
- W2027360988
- Cited by
- 257
- References
- 0
- FWCI
- 4.84
- Type
- journal article
- Language
- en
- Open access
- bronze
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- CoChat. (2026). Citation check: A retinitis pigmentosa GTPase regulator (RPGR)- deficient mouse model for X-linked retinitis pigmentosa (RP3). https://app.cochat.ai/papers/10.1073/pnas.97.7.3649
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- Hong, D.-H., Pawlyk, B. S., Shang, J., Sandberg, M. A., Berson, E. L., & Li, T. (2000). A retinitis pigmentosa GTPase regulator (RPGR)- deficient mouse model for X-linked retinitis pigmentosa (RP3). Proceedings of the National Academy of Sciences. https://doi.org/10.1073/pnas.97.7.3649
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Contains information from the Semantic Scholar Academic Graph Datasets, made available under the ODC Attribution License (ODC-BY 1.0).
Semantic Scholar’s data is described in: Kinney, R., et al. (2023). The Semantic Scholar Open Data Platform. arXiv:2301.10140.
