ArticleHuman molecular genetics2010
The retinitis pigmentosa protein RP2 interacts with polycystin 2 and regulates cilia-mediated vertebrate development.
Article in Human molecular genetics, 2010. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 40 papers.
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Who cites it
40 citing papers in PubMed, 64 citations in OpenAlex.
- Article
- Computational design and genetic incorporation of lipidation mimics in living cells.Nature chemical biology · 2024Article
- Comprehensive Analysis of Transcriptional Expression of hsa-mir-21 Predicted Target Genes and Immune Characteristics in Kidney Renal Clear Cell Carcinoma.International journal of medical sciences · 2022Article
- Establishing and regulating the composition of cilia for signal transduction.Nature reviews. Molecular cell biology · 2019Review
- Characterization of a novel RP2-OSTF1 interaction and its implication for actin remodelling.Journal of cell science · 2018Article
- Arl3 and RP2 regulate the trafficking of ciliary tip kinesins.Human molecular genetics · 2017Article
- Pathogenic mutations in retinitis pigmentosa 2 predominantly result in loss of RP2 protein stability in humans and zebrafish.The Journal of biological chemistry · 2017Article
- Identification and Expression Analysis of the Complete Family of ZebrafishFrontiers in cell and developmental biology · 2017Article
- Using induced pluripotent stem cells to understand retinal ciliopathy disease mechanisms and develop therapies.Biochemical Society transactions · 2016Review
- Sorting of lipidated cargo by the Arl2/Arl3 system.Small GTPases · 2016Review
- Novel Biochemical and Structural Insights into the Interaction of Myristoylated Cargo with Unc119 Protein and Their Release by Arl2/3.The Journal of biological chemistry · 2016Article
- PDE6δ-mediated sorting of INPP5E into the cilium is determined by cargo-carrier affinity.Nature communications · 2016Article
- Ciliary Extracellular Vesicles: Txt Msg Organelles.Cellular and molecular neurobiology · 2016Review
- Zebrafish as a Model for Human Ciliopathies.Journal of genetics and genomics = Yi chuan xue bao · 2016Review
- Methods for Studying Ciliary Import Mechanisms.Methods in molecular biology (Clifton, N.J.) · 2016Article
- Loss of retinitis pigmentosa 2 (RP2) protein affects cone photoreceptor sensory cilium elongation in mice.Cytoskeleton (Hoboken, N.J.) · 2015Article
- SUMOylation regulates ciliary localization of olfactory signaling proteins.Journal of cell science · 2015Article
- Genotype-phenotype correlation and mutation spectrum in a large cohort of patients with inherited retinal dystrophy revealed by next-generation sequencing.Genetics in medicine : official journal of the American College of Medical Genetics · 2015Article
- Mistrafficking of prenylated proteins causes retinitis pigmentosa 2.FASEB journal : official publication of the Federation of American Societies for Experimental Biology · 2015Article
- Translational read-through of the RP2 Arg120stop mutation in patient iPSC-derived retinal pigment epithelium cells.Human molecular genetics · 2015Article
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Authors and funding
9 authors at 3 institutions in 2 countries.
Funding
Abstract
Ciliopathies represent a growing group of human genetic diseases whose etiology lies in defects in ciliogenesis or ciliary function. Given the established entity of renal-retinal ciliopathies, we have been examining the role of cilia-localized proteins mutated in retinitis pigmentosa (RP) in regulating renal ciliogenesis or cilia-dependent signaling cascades. Specifically, this study examines the role of the RP2 gene product with an emphasis on renal and vertebrate development. We demonstrate that in renal epithelia, RP2 localizes to the primary cilium through dual acylation of the amino-terminus. We also show that RP2 forms a calcium-sensitive complex with the autosomal dominant polycystic kidney disease protein polycystin 2. Ablation of RP2 by shRNA promotes swelling of the cilia tip that may be a result of aberrant trafficking of polycystin 2 and other ciliary proteins. Morpholino-mediated repression of RP2 expression in zebrafish results in multiple developmental defects that have been previously associated with ciliary dysfunction, such as hydrocephalus, kidney cysts and situs inversus. Finally, we demonstrate that, in addition to our observed physical interaction between RP2 and polycystin 2, dual morpholino-mediated knockdown of polycystin 2 and RP2 results in enhanced situs inversus, indicating that these two genes also regulate a common developmental process. This work suggests that RP2 may be an important regulator of ciliary function through its association with polycystin 2 and provides evidence of a further link between retinal and renal cilia function.
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