ArticleProceedings of the National Academy of Sciences of the United States of America2004
Knock-in human rhodopsin-GFP fusions as mouse models for human disease and targets for gene therapy.
Article in Proceedings of the National Academy of Sciences of the United States of America, 2004. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 57 papers.
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The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.
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Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.
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Who cites it
57 citing papers in PubMed, 88 citations in OpenAlex.
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- Rhodopsin mislocalization drives ciliary dysregulation in a novel autosomal dominant retinitis pigmentosa knock-in mouse model.FASEB journal : official publication of the Federation of American Societies for Experimental Biology · 2024Article
- Light regulation of rhodopsin distribution during outer segment renewal in murine rod photoreceptors.Current biology : CB · 2024Article
- Super-resolution mapping in rod photoreceptors identifies rhodopsin trafficking through the inner segment plasma membrane as an essential subcellular pathway.PLoS biology · 2024Article
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- Subcellular localization of mutant P23H rhodopsin in an RFP fusion knock-in mouse model of retinitis pigmentosa.Disease models & mechanisms · 2022Article
- CRISPR genome surgery in a novel humanized model for autosomal dominant retinitis pigmentosa.Molecular therapy : the journal of the American Society of Gene Therapy · 2022Article
- Visualization of real-time receptor endocytosis in dopamine neurons enabled by NTSR1-Venus knock-in mice.Frontiers in cellular neuroscience · 2022Article
- Knock-In Mouse Models to Investigate the Functions of Opioid ReceptorsFrontiers in cellular neuroscience · 2022Review
- An N-terminal fusion allele to study melanin concentrating hormone receptor 1.Genesis (New York, N.Y. : 2000) · 2021Article
- HIF-1α and HIF-2α redundantly promote retinal neovascularization in patients with ischemic retinal disease.The Journal of clinical investigation · 2021Article
- In vivo mapping of a GPCR interactome using knockin mice.Proceedings of the National Academy of Sciences of the United States of America · 2020Article
- The Y227N mutation affects bestrophin-1 protein stability and impairs sperm function in a mouse model of Best vitelliform macular dystrophy.Biology open · 2019Article
- The route of the visual receptor rhodopsin along the cilium.Journal of cell science · 2019Article
- Humanising the mouse genome piece by piece.Nature communications · 2019Review
- A Novel Mouse Model ofFrontiers in neuroscience · 2019Article
- Two-photon imaging of the mammalian retina with ultrafast pulsing laser.JCI insight · 2018Article
- Aberrant protein trafficking in retinal degenerations: The initial phase of retinal remodeling.Experimental eye research · 2016Review
Corrections and comments
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Authors and funding
4 authors at 1 institution in 1 country.
Funding
Abstract
The human rhodopsin gene is the locus for numerous alleles linked to the neurodegenerative disease retinitis pigmentosa. To facilitate the study of retinal degeneration and to test reagents designed to alter the structure and function of this gene, we have developed strains of mice whose native rhodopsin gene has been replaced with the corresponding human DNA modified to encode an enhanced GFP fusion at the C terminus of rhodopsin. The human rhodopsin-GFP fusion faithfully mimics the expression and distribution of wild-type rhodopsin in heterozygotes and serves as a sensitive reporter of rod-cell structure and integrity. In homozygotes, however, the gene induces progressive retinal degeneration bearing many of the hallmarks of recessive retinitis pigmentosa. When the gene is flanked by recognition sites for Cre recombinase, protein expression is reduced approximately 5-fold despite undiminished mRNA levels, suggesting translation inhibition. GFP-tagged human rhodopsin provides a sensitive method to monitor the development of normal and diseased retinas in dissected samples, and it offers a noninvasive means to observe the progress of retinal degeneration and the efficacy of gene-based therapies in whole animals.
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Registered trials
Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.