ArticleScientific reports2015
Ablation of retinal ciliopathy protein RPGR results in altered photoreceptor ciliary composition.
Article in Scientific reports, 2015. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 21 papers.
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Who cites it
21 citing papers in PubMed.
- Male germ cell-associated kinase is required for axoneme formation during ciliogenesis in zebrafish photoreceptors.Disease models & mechanisms · 2024Article
- Investigating G-quadruplex structures inHeliyon · 2024Article
- Cone Structure and Function in RPGR- and USH2A-Associated Retinal Degeneration.American journal of ophthalmology · 2023Article
- Genetic Characteristics and Long-Term Follow-Up of Slovenian Patients with RPGR Retinal Dystrophy.International journal of molecular sciences · 2023Article
- Composition, organization and mechanisms of the transition zone, a gate for the cilium.EMBO reports · 2022Review
- Human iPSC-Derived Retinal Organoids and Retinal Pigment Epithelium for Novel IntronicJournal of personalized medicine · 2022Article
- Identification of a novel RPGR mutation associated with X-linked cone-rod dystrophy in a Chinese family.BMC ophthalmology · 2021Article
- RPGR isoform imbalance causes ciliary defects due to exon ORF15 mutations in X-linked retinitis pigmentosa (XLRP).Human molecular genetics · 2021Article
- Ik2/TBK1 and Hook/Dynein, an adaptor complex for early endosome transport, are genetic modifiers of FTD-associated mutant CHMP2B toxicity in Drosophila.Scientific reports · 2020Article
- Toxicity and Efficacy Evaluation of an Adeno-Associated Virus Vector Expressing Codon-OptimizedHuman gene therapy · 2020Article
- Insights into photoreceptor ciliogenesis revealed by animal models.Progress in retinal and eye research · 2019Review
- Toward genome editing in X-linked RP-development of a mouse model with specific treatment relevant features.Translational research : the journal of laboratory and clinical medicine · 2019Article
- RPGR protein complex regulates proteasome activity and mediates store-operated calcium entry.Oncotarget · 2018Article
- Prenylated retinal ciliopathy protein RPGR interacts with PDE6δ and regulates ciliary localization of Joubert syndrome-associated protein INPP5E.Human molecular genetics · 2016Article
- Ciliopathy-associated protein CEP290 modifies the severity of retinal degeneration due to loss of RPGR.Human molecular genetics · 2016Article
- The carboxyl terminal mutational hotspot of the ciliary disease protein RPGRORF15 (retinitis pigmentosa GTPase regulator) is glutamylated in vivo.Biology open · 2016Article
- Loss of human disease protein retinitis pigmentosa GTPase regulator (RPGR) differentially affects rod or cone-enriched retina.Human molecular genetics · 2016Article
- MKS5 and CEP290 Dependent Assembly Pathway of the Ciliary Transition Zone.PLoS biology · 2016Article
- Molecular studies of phenotype variation in canine RPGR-XLPRA1.Molecular vision · 2016Article
- OFD1, as a Ciliary Protein, Exhibits Neuroprotective Function in Photoreceptor Degeneration Models.PloS one · 2016Article
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4 authors.
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Abstract
Cilia regulate several developmental and homeostatic pathways that are critical to survival. Sensory cilia of photoreceptors regulate phototransduction cascade for visual processing. Mutations in the ciliary protein RPGR (retinitis pigmentosa GTPase regulator) are a prominent cause of severe blindness disorders due to degeneration of mature photoreceptors. However, precise function of RPGR is still unclear. Here we studied the involvement of RPGR in ciliary trafficking by analyzing the composition of photoreceptor sensory cilia (PSC) in Rpgr(ko) retina. Using tandem mass spectrometry analysis followed by immunoblotting, we detected few alterations in levels of proteins involved in proteasomal function and vesicular trafficking in Rpgr(ko) PSC, prior to onset of degeneration. We also found alterations in the levels of high molecular weight soluble proteins in Rpgr(ko) PSC. Our data indicate RPGR regulates entry or retention of soluble proteins in photoreceptor cilia but spares the trafficking of key structural and phototransduction-associated proteins. Given a frequent occurrence of RPGR mutations in severe photoreceptor degeneration due to ciliary disorders, our results provide insights into pathways resulting in altered mature cilia function in ciliopathies.
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