RPGR, a prenylated retinal ciliopathy protein, is targeted to cilia in a prenylation- and PDE6D-dependent manner
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چکیده
منابع مشابه
RPGR, a prenylated retinal ciliopathy protein, is targeted to cilia in a prenylation- and PDE6D-dependent manner
RPGR (retinitis pigmentosa GTPase regulator) is a ciliary protein associated with several forms of inherited retinal degenerative diseases. PDE6D is a ubiquitously expressed prenyl-binding protein and involved in ciliary targeting of prenylated proteins. The current working model for the RPGR function depicts that RPGR acts as a scaffold protein to recruit cargo-loaded PDE6D to primary cilia. H...
متن کاملPDE6D binds to the C-terminus of RPGR in a prenylation-dependent manner.
C iliary dysfunction underlies multiple human genetic diseases, and mechanisms of protein trafficking to cilia are an area of active investigation. PDE6D (also known as PDEd and PrBP) is a prenylbinding protein involved in ciliary targeting of prenylated proteins [1–4]. Previous studies uncovered that PDE6D interacts with RPGR [5,6], mutations of which cause retinitis pigmentosa [7–10]. More re...
متن کاملAblation of retinal ciliopathy protein RPGR results in altered photoreceptor ciliary composition
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 s...
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Motile cilia are an essential component of the mouse, zebrafish, and Xenopus laevis Left Right Organizers, generating nodal flow and allowing the reception and transduction of mechanosensory signals. Nonmotile primary cilia are also an important component of the Left Right Organizer's chemosensory mechanism. It has been proposed in the chicken that signaling in Hensen's node, the Left Right Org...
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We recently reported that mutations in the widely expressed nuclear protein TOPORS (topoisomerase I-binding arginine/serine rich) are associated with autosomal dominant retinal degeneration. However, the precise localization and a functional role of TOPORS in the retina remain unknown. Here, we demonstrate that TOPORS is a novel component of the photoreceptor sensory cilium, which is a modified...
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ژورنال
عنوان ژورنال: Biology Open
سال: 2016
ISSN: 2046-6390
DOI: 10.1242/bio.020461