{"id":"W4408912868","doi":"10.1101/2025.03.24.644908","title":"Characterization of a C9orf72 Knockout <i>Danio rerio</i> model for ALS and cross-species validation of potential therapeutics screened in <i>Caenorhabditis elegans</i>","year":2025,"lang":"en","type":"preprint","venue":"bioRxiv (Cold Spring Harbor Laboratory)","topic":"Amyotrophic Lateral Sclerosis Research","field":"Medicine","cited_by":0,"is_retracted":false,"has_abstract":true,"ca_institutions":"Montreal Neurological Institute and Hospital; Université de Montréal; McGill University; Centre Hospitalier de l’Université de Montréal","funders":"Canadian Institutes of Health Research; Natural Sciences and Engineering Research Council of Canada; Fondation Brain Canada; ALS Society of Canada; U.S. Department of Defense","keywords":"C9orf72; Zebrafish; Amyotrophic lateral sclerosis; Biology; Caenorhabditis elegans; Danio; Phenotype; Neurodegeneration; Genetics; Frontotemporal dementia; Trinucleotide repeat expansion; Neuroscience; Cell biology; Gene; Disease; Medicine; Pathology; Dementia; Allele","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":[0.0005673692,0.001364261,0.0008731214,0.0009479717,0.0007201954,0.0004055021,0.0008660656,0.001207227,0.00211102],"category_scores_gemma":[0.0002075484,0.0003668626,0.001081533,0.0002617213,0.0005101325,0.0003973633,0.0003823633,0.001446296,0.0008632388],"about_ca_system_candidate":false,"about_ca_system_consensus":false,"about_ca_system_score_codex":0.001050668,"about_ca_system_score_gemma":0.0009397957,"about_ca_topic_candidate":false,"about_ca_topic_consensus":false,"about_ca_topic_score_codex":0.004705904,"about_ca_topic_score_gemma":0.01346182,"domain_scores_codex":[0.9994755,0.00003721618,0.0000684004,0.0001398056,0.0001830549,0.00009606096],"domain_scores_gemma":[0.9996384,0.00003290298,0.0001195981,0.00003620305,0.0000787165,0.00009401717],"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":[0.00008216914,0.0000728725,0.00008440782,0.00005516423,0.000009222587,0.0000750758,0.00001021308,0.0000693004,0.9986934,0.00007550893,0.0001516444,0.0006211086],"study_design_scores_gemma":[0.00008012727,0.001509339,0.002973924,0.00002441135,0.00008551641,0.0002804275,0.00003424177,0.0007389478,0.9888796,0.00004083634,0.005328599,0.00002417407],"study_design_candidate":"bench_or_experimental","study_design_consensus":"bench_or_experimental","genre_codex":"empirical","genre_gemma":"empirical","genre_scores_codex":[0.9602023,0.00243734,0.01595867,0.0007844011,0.0003005892,0.001908951,0.01012909,0.001233844,0.007044831],"genre_scores_gemma":[0.9171638,0.003345297,0.03760484,0.0007173402,0.00004683368,0.002782753,0.01060263,0.0003805343,0.02735603],"genre_candidate":"empirical","genre_consensus":"empirical","teacher_disagreement_score":0.004705904,"threshold_uncertainty_score":0.009357035,"prediction_status":"machine_predicted_unvalidated"},"machine_scores":{"provisional":true,"baseline":true,"maturity_gate_passed":false,"score_opus":0.03016717489810175,"score_gpt":0.2790191322598746,"score_spread":0.2488519573617728,"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."}}