{"id":"W4411758276","doi":"10.1186/s40478-025-02061-5","title":"C9orf72 deficiency impairs the autophagic response to aggregated TDP-25 and exacerbates TDP-25-mediated neurodegeneration in vivo","year":2025,"lang":"en","type":"article","venue":"Acta Neuropathologica Communications","topic":"Amyotrophic Lateral Sclerosis Research","field":"Medicine","cited_by":7,"is_retracted":false,"has_abstract":true,"ca_institutions":"Discovery Centre; University of Toronto","funders":"Weston Brain Institute; Fondation Brain Canada; ALS Society of Canada; Canadian Institutes of Health Research; Association for Frontotemporal Degeneration","keywords":"C9orf72; Neurodegeneration; Amyotrophic lateral sclerosis; Autophagy; Frontotemporal dementia; Frontotemporal lobar degeneration; Biology; Neuroscience; Loss function; Pathogenesis; Dementia; Cell biology; Medicine; Pathology; Phenotype; Disease; Immunology; Genetics","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":[0.0003288378,0.001047129,0.000674445,0.0006126673,0.0002862001,0.0003927471,0.0003898425,0.0007707804,0.001423486],"category_scores_gemma":[0.0001382987,0.0003152189,0.0004980974,0.0001764744,0.0005628198,0.0003157917,0.000243837,0.001094498,0.0005595341],"about_ca_system_candidate":false,"about_ca_system_consensus":false,"about_ca_system_score_codex":0.0005870532,"about_ca_system_score_gemma":0.0003104802,"about_ca_topic_candidate":false,"about_ca_topic_consensus":false,"about_ca_topic_score_codex":0.001324707,"about_ca_topic_score_gemma":0.002275758,"domain_scores_codex":[0.9996194,0.00005194707,0.00004842129,0.00008647321,0.00008260622,0.0001111777],"domain_scores_gemma":[0.9997088,0.00002944994,0.00008121468,0.00002590233,0.00001793353,0.0001367321],"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":[0.0003764787,0.0001356667,0.0001080796,0.00003515715,0.00001355831,0.000134055,0.00001503377,0.0001060591,0.9985892,0.00005433886,0.00005249543,0.0003797779],"study_design_scores_gemma":[0.00007792036,0.002869413,0.005633855,0.00002160663,0.00005899911,0.0004848292,0.00005559993,0.001442997,0.9878073,0.00009558308,0.001433454,0.00001831422],"study_design_candidate":"bench_or_experimental","study_design_consensus":"bench_or_experimental","genre_codex":"empirical","genre_gemma":"empirical","genre_scores_codex":[0.9960443,0.0007401985,0.001452273,0.0000872786,0.00005135299,0.00006640465,0.0006489835,0.0001454568,0.0007638312],"genre_scores_gemma":[0.9948409,0.0004145336,0.001207831,0.00006028159,0.0000141939,0.00008206829,0.0005374551,0.00002799854,0.002814717],"genre_candidate":"empirical","genre_consensus":"empirical","teacher_disagreement_score":0.001423486,"threshold_uncertainty_score":0.004761994,"prediction_status":"machine_predicted_unvalidated"},"machine_scores":{"provisional":true,"baseline":true,"maturity_gate_passed":false,"score_opus":0.0341950351203602,"score_gpt":0.3187227231287466,"score_spread":0.2845276880083863,"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."}}