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