{"id":"W3173817036","doi":"10.1172/jci.insight.142073","title":"Early defects in mucopolysaccharidosis type IIIC disrupt excitatory synaptic transmission","year":2021,"lang":"en","type":"article","venue":"JCI Insight","topic":"Lysosomal Storage Disorders Research","field":"Medicine","cited_by":30,"is_retracted":false,"has_abstract":true,"ca_institutions":"Montreal Neurological Institute and Hospital; Université de Montréal; Institute for Research in Immunology and Cancer; McGill University; Centre Hospitalier Universitaire Sainte-Justine","funders":"Fonds de recherche du Québec – Nature et technologies; Fonds de Recherche du Québec - Santé; Fonds de recherche du Québec; Canadian Institutes of Health Research; Royal Society; Royal Society of Canada; McGill University","keywords":"Excitatory postsynaptic potential; Neurotransmission; Neuroscience; Postsynaptic potential; Neurodegeneration; Inhibitory postsynaptic potential; Dendritic spine; Synaptic vesicle; Biology; Synaptic cleft; Hippocampal formation; Synapse; Synaptic fatigue; Medicine; Pathology; Central nervous system; Neurotransmitter; Receptor; Vesicle; Biochemistry","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":["insufficient_payload"],"consensus_categories":[],"category_scores_codex":[0.0001304006,0.000179642,0.0003472069,0.0002432128,0.00007398333,0.00003288915,0.000119199,0.0001801407,0.001180919],"category_scores_gemma":[0.0002652402,0.0001573505,0.0001215357,0.001039919,0.00007178821,0.0001442863,0.00006134172,0.0004436931,0.000344112],"about_ca_system_candidate":false,"about_ca_system_consensus":false,"about_ca_system_score_codex":0.0001884833,"about_ca_system_score_gemma":0.0003859992,"about_ca_topic_candidate":false,"about_ca_topic_consensus":false,"about_ca_topic_score_codex":0.0001483394,"about_ca_topic_score_gemma":0.00006111211,"domain_scores_codex":[0.9982392,0.0001248667,0.0002736833,0.0004226113,0.0005084496,0.0004312044],"domain_scores_gemma":[0.9990326,0.0001048929,0.00003353602,0.0003859667,0.0001757827,0.0002672662],"domain_codex":null,"domain_gemma":null,"domain_candidate":null,"domain_consensus":null,"study_design_codex":"bench_or_experimental","study_design_gemma":"observational","study_design_scores_codex":[0.001569924,0.003278743,0.2517447,0.001368985,0.0004629442,0.01604881,0.01202935,0.00001891183,0.6636449,0.001570861,0.003371209,0.04489065],"study_design_scores_gemma":[0.01069724,0.002030019,0.8199838,0.001376428,0.000275804,0.0003615614,0.001263908,0.001285141,0.1007364,0.00136726,0.05947139,0.001151103],"study_design_candidate":"observational","study_design_consensus":null,"genre_codex":"empirical","genre_gemma":"empirical","genre_scores_codex":[0.9825071,0.00746507,0.0001275227,0.000742639,0.0001798689,0.0003085574,0.000001637205,0.00007038547,0.008597275],"genre_scores_gemma":[0.9970433,0.0004140886,0.0002195667,0.0004033024,0.00007262467,0.00002678337,0.00001899768,0.00004514356,0.001756245],"genre_candidate":"empirical","genre_consensus":"empirical","teacher_disagreement_score":0.568239,"threshold_uncertainty_score":0.9997321,"prediction_status":"machine_predicted_unvalidated"},"machine_scores":{"provisional":true,"baseline":true,"maturity_gate_passed":false,"score_opus":0.02577394215930377,"score_gpt":0.2969166765177952,"score_spread":0.2711427343584914,"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."}}