27 results found
    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

    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. Human Biology and Medicine
    2. Neuroscience

    Subretinal mononuclear phagocytes induce cone segment loss via IL-1β

    Chiara M Eandi et al.
    IL-1β release from macrophages might be responsible for the unexplained cone segment loss in retinal degenerative diseases that are associated with subretinal inflammation, such as retinitis pigmentosa or geographic atrophy.
    1. Biochemistry and Chemical Biology
    2. Cell Biology

    Small molecule proteostasis regulators that reprogram the ER to reduce extracellular protein aggregation

    Lars Plate et al.
    Small molecule proteostasis regulators that activate the unfolded protein response transcription factor ATF6 reduce the secretion of amyloid disease-associated proteins.
    1. Human Biology and Medicine
    2. Neuroscience

    Probing the functional impact of sub-retinal prosthesis

    Sébastien Roux et al.
    Existing artificial retinas produce distorted and imprecise activation of the visual system, but reverse engineering promises to refine the induced activation patterns.
    1. Cell Biology

    The ER membrane protein complex interacts cotranslationally to enable biogenesis of multipass membrane proteins

    Matthew J Shurtleff et al.
    The ER membrane protein complex promotes the biogenesis of a subset multipass membrane proteins enriched for transporters and other proteins with destabilizing features in transmembrane domains.
    1. Biochemistry and Chemical Biology
    2. Neuroscience

    Biochemical adaptations of the retina and retinal pigment epithelium support a metabolic ecosystem in the vertebrate eye

    Mark A Kanow et al.
    Metabolic relationships between cells in the retina and retinal pigment epithelium are fundamental to retinal function, retinal disease and age-related vision loss and they may provide strategies for metabolism-based therapies.
    1. Structural Biology and Molecular Biophysics

    Patient-specific mutations impair BESTROPHIN1’s essential role in mediating Ca2+-dependent Cl- currents in human RPE

    Yao Li et al.
    A multidisciplinary platform featured by patient-derived RPEs is established to study the disease-causing mechanisms of BEST1 mutations, and demonstrates gene-supplemented rescue of the mutation-caused deficiency in Ca2+-dependent Cl- current in human RPE.
    1. Cell Biology

    dPob/EMC is essential for biosynthesis of rhodopsin and other multi-pass membrane proteins in Drosophila photoreceptors

    Takunori Satoh et al.
    A membrane protein complex in the endoplasmic reticulum is a key factor for the biogenesis of multi-pass transmembrane proteins, including Rh1, and its loss causes retinal degeneration.

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