14 results found
    1. Neuroscience

    Small molecule Photoregulin3 prevents retinal degeneration in the RhoP23H mouse model of retinitis pigmentosa

    Paul A Nakamura et al.
    Regulating rod gene expression with a small molecule ligand for the orphan nuclear receptor Nr2e3 rescues photoreceptors from degeneration in a mouse model of retinitis pigmentosa.
    1. Biochemistry and Chemical Biology
    2. Cell Biology

    Phospholipase D activity couples plasma membrane endocytosis with retromer dependent recycling

    Rajan Thakur et al.
    Phosphatidic acid regulates recycling of endocytosed plasma membrane vesicles in polarized cells through a retromer and Arf1 dependent process.
    1. Neuroscience

    Guanylate cyclase 1 relies on rhodopsin for intracellular stability and ciliary trafficking

    Jillian N Pearring et al.
    Rather than relying on intrinsic intracellular targeting information, the key phototransduction enzyme guanylate cyclase 1 is delivered to the photosensory cilium with the visual pigment rhodopsin.
    1. Human Biology and Medicine
    2. Neuroscience

    Impaired ABCA1/ABCG1-mediated lipid efflux in the mouse retinal pigment epithelium (RPE) leads to retinal degeneration

    Federica Storti et al.
    Lipid efflux by the retinal pigment epithelium is crucial for proper retinal integrity and function, and its impairment may contribute to diseases like age-related macular degeneration.
    1. Cell Biology
    2. Neuroscience

    Hypoxia-induced metabolic stress in retinal pigment epithelial cells is sufficient to induce photoreceptor degeneration

    Toshihide Kurihara et al.
    Mouse models in which hypoxia can be genetically triggered in retinal pigmented epithelial cells show that hypoxia-induced metabolic stress alone can lead to photoreceptor atrophy/dysfunction.
    1. Genetics and Genomics
    2. Neuroscience

    Epigenomic landscapes of retinal rods and cones

    Alisa Mo et al.
    Genome-wide analysis of DNA methylation and accessible chromatin shows that retinal rods and cones have distinct epigenomic features that reflect differences in their development and function.
    1. Developmental Biology
    2. Chromosomes and Gene Expression

    Patient-specific iPSC-derived photoreceptor precursor cells as a means to investigate retinitis pigmentosa

    Budd A Tucker et al.
    Skin cells from a patient with retinitis pigmentosa have been used to generate induced pluripotent stem cells, which could potentially form the basis of new treatments for this disease.
    1. Neuroscience

    Dystroglycan is a scaffold for extracellular axon guidance decisions

    L Bailey Lindenmaier et al.
    Dystroglycan interacts with multiple partners, including the transmembrane receptor Celsr3, to regulate axon tract formation throughout the developing nervous system.
    1. Cell Biology

    Dynein-2 intermediate chains play crucial but distinct roles in primary cilia formation and function

    Laura Vuolo et al.
    Genetic knock-outs of the dynein-2 intermediate chains reveals that both are essential for correct cilia function and transition zone organization, but play different functions in the assembly of dynein-2 motor and in primary cilia formation.
    1. Biochemistry and Chemical Biology
    2. Structural Biology and Molecular Biophysics

    A recombinant BBSome core complex and how it interacts with ciliary cargo

    Björn Udo Klink et al.
    Six BBS proteins form a core BBSome transport vehicle, which is sufficient for recognizing membrane proteins for transport into the ciliary compartment.

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