{"id":"W4317937265","doi":"10.1016/j.celrep.2023.112025","title":"FUS ALS neurons activate major stress pathways and reduce translation as an early protective mechanism against neurodegeneration","year":2023,"lang":"en","type":"article","venue":"Cell Reports","topic":"Amyotrophic Lateral Sclerosis Research","field":"Medicine","cited_by":65,"is_retracted":false,"has_abstract":true,"ca_institutions":"University of Toronto","funders":"Deutsche Gesellschaft für Muskelkranke; NOMIS Stiftung; Canada First Research Excellence Fund; Canada Research Chairs; Technische Universität Dresden; Hermann und Lilly Schilling-Stiftung für Medizinische Forschung; Eli Lilly and Company","keywords":"Stress granule; Neurodegeneration; Amyotrophic lateral sclerosis; Cell biology; Translation (biology); C9orf72; Proteotoxicity; Biology; Mutant; Downregulation and upregulation; Unfolded protein response; Neuroscience; Chemistry; Protein aggregation; Endoplasmic reticulum; Biochemistry; Medicine; Messenger RNA; Trinucleotide repeat expansion; Pathology; Gene","routes":{"ca_aff":true,"ca_fund":true,"ca_venue":false,"about_ca":false,"invisible_to_affiliation_only":false},"retraction":null,"screen":null,"direct_labels":[],"prediction":{"model_version":"codex-gemma-dda1882f352a","candidate_categories":[],"consensus_categories":[],"category_scores_codex":[0.0003213667,0.0001839549,0.0002273274,0.0001617037,0.0001910585,0.0000794659,0.00005073872,0.0001352801,0.00003324585],"category_scores_gemma":[0.0001028712,0.0001628004,0.00005458717,0.0003280649,0.00004851338,0.0002849673,0.00004517706,0.0003063838,0.0000230632],"about_ca_system_candidate":false,"about_ca_system_consensus":false,"about_ca_system_score_codex":0.00005172665,"about_ca_system_score_gemma":0.000125859,"about_ca_topic_candidate":false,"about_ca_topic_consensus":false,"about_ca_topic_score_codex":0.0001747962,"about_ca_topic_score_gemma":0.00001284451,"domain_scores_codex":[0.9980091,0.0001506418,0.0003512703,0.0006227266,0.000512523,0.0003537321],"domain_scores_gemma":[0.9989806,0.00003455437,0.0001522493,0.0004271778,0.0001298618,0.0002755167],"domain_codex":null,"domain_gemma":null,"domain_candidate":null,"domain_consensus":null,"study_design_codex":"bench_or_experimental","study_design_gemma":"bench_or_experimental","study_design_scores_codex":[0.000104996,0.00008189592,0.0005914381,0.00005941137,0.00001355504,0.001312595,0.0007101632,0.00006562996,0.9863098,0.00003230136,0.00003405649,0.01068413],"study_design_scores_gemma":[0.0007068382,0.0008154944,0.1274638,0.00007542396,0.00004079344,0.0001350173,0.00008589854,0.002576659,0.8662748,0.001501592,0.0001098174,0.0002139037],"study_design_candidate":"bench_or_experimental","study_design_consensus":"bench_or_experimental","genre_codex":"empirical","genre_gemma":"empirical","genre_scores_codex":[0.9961693,0.00002649083,0.000155224,0.0005681522,0.0001696598,0.001485055,0.000009122525,0.0002239527,0.00119302],"genre_scores_gemma":[0.9982938,0.00008985624,0.000157635,0.00008653005,0.0001581707,0.0001487477,0.000119864,0.00005685789,0.0008885343],"genre_candidate":"empirical","genre_consensus":"empirical","teacher_disagreement_score":0.1268723,"threshold_uncertainty_score":0.6638806,"prediction_status":"machine_predicted_unvalidated"},"machine_scores":{"provisional":true,"baseline":true,"maturity_gate_passed":false,"score_opus":0.04857849395920638,"score_gpt":0.2905731305183171,"score_spread":0.2419946365591107,"validation_status":"score_only:v0-immature-baseline","note":"Baseline scores from an immature model (maturity gate not passed). Scores rank; they never assert a category."}}