35 results found
    1. Neuroscience

    Suppression of C9orf72 RNA repeat-induced neurotoxicity by the ALS-associated RNA-binding protein Zfp106

    Barbara Celona et al.
    Zfp106 functions as an RNA binding protein, binds directly to GGGGCC RNA repeats, is required in motor neurons to prevent ALS-like neurodegeneration in mice, and can suppress neurotoxicity in an established fly model of ALS.
    1. Cancer Biology
    2. Neuroscience

    Pharmacological augmentation of nicotinamide phosphoribosyltransferase (NAMPT) protects against paclitaxel-induced peripheral neuropathy

    Peter M LoCoco et al.
    Stimulation of the rate-limiting enzyme in the NAD salvage pathway protected against neurotoxicity and subsequent peripheral neuropathy associated with the widely utilized anticancer drug, paclitaxel.
    1. Cell Biology
    2. Neuroscience

    Matrin 3-dependent neurotoxicity is modified by nucleic acid binding and nucleocytoplasmic localization

    Ahmed M Malik et al.
    The deposition of Matrin 3, a DNA/RNA binding protein implicated in ALS and FTD, results in neurodegeneration dependent upon its localization, self-association, and ability to bind nucleic acids.
    1. Neuroscience

    Increased expression of heme-binding protein 1 early in Alzheimer's disease is linked to neurotoxicity

    Oleksandr Yagensky et al.
    Increased levels of brain Hebp1 starting from the presymptomatic stage of Alzheimer’s disease contributes to progressive neuronal loss by triggering mitochondrial-dependent apoptosis in neurons exposed to elevated heme.
    1. Neuroscience

    The N-terminus of the prion protein is a toxic effector regulated by the C-terminus

    Bei Wu et al.
    A novel auto-inhibitory mechanism regulates the functional activity of the cellular prion protein, PrPC, providing for the first time a coherent molecular model for both its pathological and physiological effects.
    1. Biochemistry and Chemical Biology
    2. Cell Biology

    Control of the structural landscape and neuronal proteotoxicity of mutant Huntingtin by domains flanking the polyQ tract

    Koning Shen et al.
    The polyQ tract of pathogenic Huntingtin causes aggregation when expanded in Huntington’s disease, but its two flanking domains control its conformational landscape, proteostasis and neurotoxicity.
    1. Neuroscience

    Energy imbalance alters Ca2+ handling and excitability of POMC neurons

    Lars Paeger et al.
    The development of diet-dependent obesity results in the deterioration of calcium homeostasis in pro-opiomelanocortin-expressing neurons and impaired function of the same neurons.
    1. Human Biology and Medicine
    2. Neuroscience

    Rett-causing mutations reveal two domains critical for MeCP2 function and for toxicity in MECP2 duplication syndrome mice

    Laura Dean Heckman et al.
    Transgenic mice with Rett-causing mutations in MeCP2 reveal that a basic cluster in the C-terminus of the protein binds DNA and that both the methyl-CpG binding domain and the transcriptional repression domain are necessary to elicit toxicity in MECP2 duplication syndrome.
    1. Neuroscience

    The Ret receptor regulates sensory neuron dendrite growth and integrin mediated adhesion

    Peter Soba et al.
    A cell surface receptor called Ret enables neurons to establish and maintain the organized arrays of dendrites that support communication between neurons.
    1. Chromosomes and Gene Expression
    2. Human Biology and Medicine

    Trisomy 21 consistently activates the interferon response

    Kelly D Sullivan et al.
    Hyperactive interferon signaling is a hallmark of trisomy 21 and may contribute to many of the comorbidities associated with Down syndrome.

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