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• W2942673887 abstract "Misfolded protein toxicity and failure of protein quality control underlie neurodegenerative diseases including amyotrophic lateral sclerosis and frontotemporal dementia. Here, we identified Lethal(3)malignant brain tumor-like protein 1 (L3MBTL1) as a key regulator of protein quality control, the loss of which protected against the proteotoxicity of mutant Cu/Zn superoxide dismutase or C9orf72 dipeptide repeat proteins. L3MBTL1 acts by regulating p53-dependent quality control systems that degrade misfolded proteins. SET domain-containing protein 8, an L3MBTL1-associated p53-binding protein, also regulated clearance of misfolded proteins and was increased by proteotoxicity-associated stresses in mammalian cells. Both L3MBTL1 and SET domain-containing protein 8 were upregulated in the central nervous systems of mouse models of amyotrophic lateral sclerosis and human patients with amyotrophic lateral sclerosis/frontotemporal dementia. The role of L3MBTL1 in protein quality control is conserved from Caenorhabditis elegans to mammalian neurons. These results reveal a protein quality-control pathway that operates in both normal stress response and proteotoxicity-associated neurodegenerative diseases. 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." @default.
• W2942673887 created "2019-05-09" @default.
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• W2942673887 date "2019-05-06" @default.
• W2942673887 modified "2023-10-01" @default.
• W2942673887 title "L3MBTL1 regulates ALS/FTD-associated proteotoxicity and quality control" @default.
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• W2942673887 doi "https://doi.org/10.1038/s41593-019-0384-5" @default.
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