{"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":"metacan-v3-hybrid-931329e0061c","candidate_categories":[],"consensus_categories":[],"category_scores_codex":[0.0001137431,0.0005752548,0.0003728218,0.0001802157,0.0001680822,0.0002954449,0.0001377934,0.0003078238,0.001573313],"category_scores_gemma":[0.00008062755,0.0001046064,0.0003229293,0.00009959412,0.0002097495,0.000270434,0.0003295898,0.000400796,0.0003466771],"about_ca_system_candidate":false,"about_ca_system_consensus":false,"about_ca_system_score_codex":0.0002953035,"about_ca_system_score_gemma":0.0001317668,"about_ca_topic_candidate":false,"about_ca_topic_consensus":false,"about_ca_topic_score_codex":0.0002959673,"about_ca_topic_score_gemma":0.0004341735,"domain_scores_codex":[0.9998686,0.00001285155,0.0000157795,0.00002899185,0.00004273618,0.00003098978],"domain_scores_gemma":[0.9999321,0.000007092922,0.00002198491,0.000007605596,0.00001143402,0.00001981131],"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":[0.00006856665,0.000008450149,0.0001603614,0.00003559247,0.00000400914,0.000042899,0.000008716373,0.00001709564,0.9988424,0.00005664093,0.00002621738,0.000729043],"study_design_scores_gemma":[0.0000129743,0.0003643569,0.01214473,0.00001256875,0.00001839289,0.0003631924,0.00005750115,0.0002707567,0.984871,0.000161454,0.00171806,0.000004934174],"study_design_candidate":"bench_or_experimental","study_design_consensus":"bench_or_experimental","genre_codex":"empirical","genre_gemma":"empirical","genre_scores_codex":[0.9913311,0.002711699,0.002680833,0.0001307079,0.00005783551,0.00003305462,0.0005694116,0.0001971834,0.002288287],"genre_scores_gemma":[0.9944955,0.001151749,0.001402337,0.00009784116,0.00001430385,0.00004662626,0.0005103936,0.00002314361,0.002258079],"genre_candidate":"empirical","genre_consensus":"empirical","teacher_disagreement_score":0.001573313,"threshold_uncertainty_score":0.005263269,"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."}}