559 results found
    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. Immunology and Inflammation
    2. Neuroscience

    CSF1R blockade induces macrophage ablation and results in mouse choroidal vascular atrophy and RPE disorganization

    Xiao Yang et al.
    Vascular degeneration of the choroid and RPE disorganization were associated with pharmacological macrophage ablation, indicating that insufficiency of macrophage function may be a mechanism underlying age- and AMD-associated pathology.
    1. Structural Biology and Molecular Biophysics
    2. Cell Biology

    Kinesin-4 KIF21B is a potent microtubule pausing factor

    Wilhelmina E van Riel et al.
    Kinesin-4 KIF21B combines a processive motor activity with two non-motor microtubule-binding domains and an autoregulatory region to induce pausing of microtubule plus ends.
    1. Cell Biology

    A signal capture and proofreading mechanism for the KDEL-receptor explains selectivity and dynamic range in ER retrieval

    Andreas Gerondopoulos et al.
    KDEL receptor structures with variant HDEL and RDEL signals help decode the cell biological mechanism for the selective retrieval of escaped endoplasmic reticulum luminal proteins.
    1. Genetics and Genomics
    2. Immunology and Inflammation

    PACT-mediated PKR activation acts as a hyperosmotic stress intensity sensor weakening osmoadaptation and enhancing inflammation

    Kenneth T Farabaugh et al.
    Changes in the interactome of the Rel family of transcription factors control adaptation to the environmental stress of hyperosmolarity and determine cell fate.
    1. 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. Structural Biology and Molecular Biophysics

    Distinct expression requirements and rescue strategies for BEST1 loss- and gain-of-function mutations

    Qingqing Zhao et al.
    BEST1 autosomal dominant loss-of-function mutations surprisingly behave in a dominant-negative manner, whereas gain-of-function mutations exhibit a strong dominant effect that necessitates CRISPR/Cas9-mediated suppression of the endogenous mutant allele in gene therapy.
    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. Computational and Systems Biology
    2. Developmental Biology

    Retinal stem cells modulate proliferative parameters to coordinate post-embryonic morphogenesis in the eye of fish

    Erika Tsingos et al.
    3D niche topology imposes a spatially biased random stem cell loss, which is differentially fine-tuned in neural retina and retinal pigmented epithelium to regulate growth, shape, and cellular topology.
    1. Developmental Biology
    2. Neuroscience

    Neural retina-specific Aldh1a1 controls dorsal choroidal vascular development via Sox9 expression in retinal pigment epithelial cells

    So Goto et al.
    Neural retina-derived retinoic acids synthesized by Aldh1a1 control choroidal vascular development by regulating RPE-secreted VEGF.

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