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LONRF2 is a protein quality control ubiquitin ligase whose deficiency causes late-onset neurological deficits
Description
Protein misfolding is a major factor of neurodegenerative diseases. Post-mitotic neurons are highly susceptible to protein aggregates that are not diluted by mitosis. Therefore, post-mitotic cells may have a specific protein quality control system. Here, we show that LONRF2 is a bona fide protein quality control ubiquitin ligase induced in post-mitotic senescent cells. Under unperturbed conditions, LONRF2 is predominantly expressed in neurons. LONRF2 binds and ubiquitylates abnormally structured TDP-43 and hnRNP M1 and artificially misfolded proteins. Lonrf2
Journal
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- Nature Aging
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Nature Aging 3 (8), 1001-1019, 2023-07-20
Springer Science and Business Media LLC
