{"id":"W2933308068","doi":"10.1007/s00401-019-01999-w","title":"ADAR2 mislocalization and widespread RNA editing aberrations in C9orf72-mediated ALS/FTD","year":2019,"lang":"en","type":"article","venue":"Acta Neuropathologica","topic":"RNA regulation and disease","field":"Biochemistry, Genetics and Molecular Biology","cited_by":70,"is_retracted":false,"has_abstract":false,"ca_institutions":"Occupational Cancer Research Centre; University of Toronto","funders":"Harrington Discovery Institute, University Hospitals; National Institute of Neurological Disorders and Stroke; Tau Consortium; National Institutes of Health; Bruno and Ilse Frick Foundation for Research on ALS; Barrow Neurological Foundation; Donald E. and Delia B. Baxter Foundation; Robert Packard Center for ALS Research, Johns Hopkins University; Association for Frontotemporal Degeneration; Southern California Clinical and Translational Science Institute; New York Stem Cell Foundation; Alzheimer's Drug Discovery Foundation; Muscular Dystrophy Association; ALS Association; Keck School of Medicine of USC; Mayo Clinic; Target ALS; Judith and Jean Pape Adams Charitable Foundation; U.S. Department of Defense","keywords":"C9orf72; RNA editing; RNA; Biology; Amyotrophic lateral sclerosis; RNA-binding protein; ADAR; Frontotemporal dementia; Induced pluripotent stem cell; Cell biology; Neuroscience; Genetics; Trinucleotide repeat expansion; Gene; Medicine; Pathology; Embryonic stem cell","routes":{"ca_aff":true,"ca_fund":false,"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":[0.0002811302,0.0007204016,0.0004612538,0.001038586,0.0005959992,0.0004393102,0.0004338957,0.0009459908,0.004104895],"category_scores_gemma":[0.0003336476,0.0002873252,0.0004329741,0.0004148631,0.0007892746,0.0002529441,0.0004364665,0.0006521589,0.0008649646],"about_ca_system_candidate":false,"about_ca_system_consensus":false,"about_ca_system_score_codex":0.0003685795,"about_ca_system_score_gemma":0.0002181603,"about_ca_topic_candidate":false,"about_ca_topic_consensus":false,"about_ca_topic_score_codex":0.001289065,"about_ca_topic_score_gemma":0.001414538,"domain_scores_codex":[0.999656,0.00003446678,0.0000458896,0.0001353017,0.00007558279,0.00005287954],"domain_scores_gemma":[0.9997278,0.00003880709,0.0001166836,0.00003030294,0.00002852921,0.00005794262],"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":[0.001271436,0.0001259133,0.006500473,0.0001030009,0.0001073736,0.03156467,0.0001478513,0.0004270588,0.9534702,0.0003584264,0.0004733728,0.005450166],"study_design_scores_gemma":[0.0002126898,0.0007392306,0.241662,0.00005847095,0.0005507328,0.2023565,0.000587615,0.004864619,0.5398112,0.001505131,0.007544,0.0001078351],"study_design_candidate":"bench_or_experimental","study_design_consensus":"bench_or_experimental","genre_codex":"empirical","genre_gemma":"empirical","genre_scores_codex":[0.9942985,0.0007229929,0.002385968,0.0001332149,0.00004889393,0.00001671461,0.0005208678,0.00015707,0.001715685],"genre_scores_gemma":[0.9981076,0.0001243591,0.0005534667,0.00003756484,0.00001342705,0.000007918463,0.0001674646,0.00002481635,0.0009633916],"genre_candidate":"empirical","genre_consensus":"empirical","teacher_disagreement_score":0.004104895,"threshold_uncertainty_score":0.01373219,"prediction_status":"machine_predicted_unvalidated"},"machine_scores":{"provisional":true,"baseline":true,"maturity_gate_passed":false,"score_opus":0.0079131552580627,"score_gpt":0.2266763433191828,"score_spread":0.2187631880611202,"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."}}