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Showing 1–3 of 3 results
Advanced filters: Author: Goran Periz Clear advanced filters

  • Lu et al. report a pathway that reprograms protein quality control under stress. Identified in a Caenorhabditis elegans screen and characterized in mammalian systems, L3MBTL1 and its partner SETD8 modulate proteotoxicity and are deregulated in patients with ALS/FTD.

    • Jiayin Lu
    • Goran Periz
    • Jiou Wang
    Research
    Nature Neuroscience
    Volume: 22, P: 875-886

  • Structurally polymorphic C9orf72 hexanucleotide repeats cause an impairment in transcriptional processivity and lead to accumulation of truncated repeat-containing transcripts that bind to specific ribonucleoproteins, such as nucleolin, in a conformation-dependent manner resulting in nucleolar stress and C9orf72-linked pathology in amyotrophic lateral sclerosis and frontotemporal dementia.

    • Aaron R. Haeusler
    • Christopher J. Donnelly
    • Jiou Wang
    Research
    Nature
    Volume: 507, P: 195-200