336 results found
    1. Neuroscience

    Homeostatic plasticity in the retina is associated with maintenance of night vision during retinal degenerative disease

    Henri Leinonen, Nguyen C Pham ... Frans Vinberg
    Mouse retina manifests homeostatic plasticity and adapts to photoreceptor degeneration to resist visual decline.
    1. Medicine

    A single-cell atlas depicting the cellular and molecular features in human anterior cruciate ligamental degeneration: A single cell combined spatial transcriptomics study

    Runze Yang, Tianhao Xu ... Weili Fu
    Single-cell combined spatial transcriptomics provides the molecular foundation for investigating how ligamental cell identities, biochemical functions, and interactions contributed to the ligamental degeneration process.
    1. Neuroscience

    Valosin-containing protein (VCP/p97) inhibitors relieve Mitofusin-dependent mitochondrial defects due to VCP disease mutants

    Ting Zhang, Prashant Mishra ... Ming Guo
    A potential medication has been found for the brain and muscle degenerative diseases caused by mutant forms of Valosin-Containing Protein.
    1. Cell Biology
    2. Neuroscience

    VCP-dependent muscle degeneration is linked to defects in a dynamic tubular lysosomal network in vivo

    Alyssa E Johnson, Huidy Shu ... Graeme W Davis
    The AAA-ATPase VCP sustains sarcoplasmic proteostasis, in part, by controlling autophagosome-lysosome fusion and the integrity of a dynamic tubular lysosomal network.
    1. Neuroscience

    Impact of long- and short-range fibre depletion on the cognitive deficits of fronto-temporal dementia

    Melissa Savard, Tharick A Pascoal ... Pedro Rosa-Neto
    While semantic symptoms in fronto-temporal dementia patients were mainly dependent on short-range white-matter fibre disruption, long-range white-matter fibres damage was the major contributor to executive dysfunction, highlighting the importance of controlling for risk factors associated with deep white-matter disease.
    1. Cell Biology
    2. Medicine

    Senotherapeutic drugs for human intervertebral disc degeneration and low back pain

    Hosni Cherif, Daniel G Bisson ... Lisbet Haglund
    Targeting senescent cells in human intervertebral discs, using senotherapeutics, provides a potential therapeutic strategy to prevent or reduce disc degeneration and pain.
    1. Structural Biology and Molecular Biophysics

    A Dynamic molecular basis for malfunction in disease mutants of p97/VCP

    Anne K Schuetz, Lewis E Kay
    Point mutations in a ubiquitous human ATPase called p97/VCP deregulate inter-domain communication, resulting in impaired binding of an adaptor that recruits p97 to endosomal pathways and leading to a degenerative disease of bone, muscle and neurons.
    1. Cell Biology
    2. Neuroscience

    Modeling of axonal endoplasmic reticulum network by spastic paraplegia proteins

    Belgin Yalçın, Lu Zhao ... Cahir J O'Kane
    Proteins of the reticulon and REEP families, homologous to the products of human Hereditary Spastic Paraplegia disease genes, contribute to shaping and continuity of the axonal endoplasmic reticulum network in Drosophila.
    1. Biochemistry and Chemical Biology

    Mitochondrial protein import clogging as a mechanism of disease

    Liam P Coyne, Xiaowen Wang ... Xin Jie Chen
    Missense mutations in the nuclear-encoded adenine nucleotide translocase 1 (Ant1) cause the protein to clog the mitochondrial protein import pathway, to severely inhibit cell growth in yeast, and to cause neurodegeneration and myopathy in mice that phenocopy ANT1-induced human disease.
    1. Neuroscience

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

    Paul A Nakamura, Andy A Shimchuk ... Thomas A Reh
    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.

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