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http://purl.uniprot.org/citations/31061493http://www.w3.org/1999/02/22-rdf-syntax-ns#typehttp://purl.uniprot.org/core/Journal_Citation
http://purl.uniprot.org/citations/31061493http://www.w3.org/2000/01/rdf-schema#comment"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."xsd:string
http://purl.uniprot.org/citations/31061493http://purl.org/dc/terms/identifier"doi:10.1038/s41593-019-0384-5"xsd:string
http://purl.uniprot.org/citations/31061493http://purl.uniprot.org/core/author"Ji Y."xsd:string
http://purl.uniprot.org/citations/31061493http://purl.uniprot.org/core/author"Liu Y."xsd:string
http://purl.uniprot.org/citations/31061493http://purl.uniprot.org/core/author"Lu J."xsd:string
http://purl.uniprot.org/citations/31061493http://purl.uniprot.org/core/author"Li W."xsd:string
http://purl.uniprot.org/citations/31061493http://purl.uniprot.org/core/author"Wang J."xsd:string
http://purl.uniprot.org/citations/31061493http://purl.uniprot.org/core/author"Zhang T."xsd:string
http://purl.uniprot.org/citations/31061493http://purl.uniprot.org/core/author"Tang Q."xsd:string
http://purl.uniprot.org/citations/31061493http://purl.uniprot.org/core/author"Johnson K."xsd:string
http://purl.uniprot.org/citations/31061493http://purl.uniprot.org/core/author"Mojsilovic-Petrovic J."xsd:string
http://purl.uniprot.org/citations/31061493http://purl.uniprot.org/core/author"Lu Y.N."xsd:string
http://purl.uniprot.org/citations/31061493http://purl.uniprot.org/core/author"Periz G."xsd:string
http://purl.uniprot.org/citations/31061493http://purl.uniprot.org/core/author"Kalb R."xsd:string
http://purl.uniprot.org/citations/31061493http://purl.uniprot.org/core/author"Thombre R."xsd:string
http://purl.uniprot.org/citations/31061493http://purl.uniprot.org/core/author"Shah Y."xsd:string
http://purl.uniprot.org/citations/31061493http://purl.uniprot.org/core/author"Aljumaah R."xsd:string
http://purl.uniprot.org/citations/31061493http://purl.uniprot.org/core/date"2019"xsd:gYear
http://purl.uniprot.org/citations/31061493http://purl.uniprot.org/core/name"Nat Neurosci"xsd:string
http://purl.uniprot.org/citations/31061493http://purl.uniprot.org/core/pages"875-886"xsd:string
http://purl.uniprot.org/citations/31061493http://purl.uniprot.org/core/title"L3MBTL1 regulates ALS/FTD-associated proteotoxicity and quality control."xsd:string
http://purl.uniprot.org/citations/31061493http://purl.uniprot.org/core/volume"22"xsd:string
http://purl.uniprot.org/citations/31061493http://www.w3.org/2004/02/skos/core#exactMatchhttp://purl.uniprot.org/pubmed/31061493
http://purl.uniprot.org/citations/31061493http://xmlns.com/foaf/0.1/primaryTopicOfhttps://pubmed.ncbi.nlm.nih.gov/31061493