{"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":"metacan-v3-hybrid-931329e0061c","candidate_categories":[],"consensus_categories":[],"category_scores_codex":[0.00009278239,0.0005248536,0.0002498092,0.0002854442,0.0001516071,0.0001840976,0.0001316782,0.0002464513,0.001083715],"category_scores_gemma":[0.0001628851,0.0001186164,0.0001943454,0.0001499256,0.0002371238,0.0001450425,0.0002423927,0.0002843252,0.0002688597],"about_ca_system_candidate":false,"about_ca_system_consensus":false,"about_ca_system_score_codex":0.0002702796,"about_ca_system_score_gemma":0.000208007,"about_ca_topic_candidate":false,"about_ca_topic_consensus":false,"about_ca_topic_score_codex":0.001197109,"about_ca_topic_score_gemma":0.002323212,"domain_scores_codex":[0.9999467,0.000006981567,0.00001054811,0.000009904476,0.00001389554,0.00001201631],"domain_scores_gemma":[0.9999259,0.00001086164,0.00002902632,0.00000714936,0.000007596836,0.0000194749],"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":[0.0001780674,0.0000396414,0.00508385,0.0001089153,0.00002806073,0.001380189,0.00004762206,0.00009750803,0.9861361,0.0001808308,0.0001908861,0.006528265],"study_design_scores_gemma":[0.00006676482,0.00121916,0.2490404,0.0001002065,0.00009348896,0.02173071,0.000261659,0.0006139347,0.717983,0.0005263348,0.008342532,0.00002182968],"study_design_candidate":"bench_or_experimental","study_design_consensus":"bench_or_experimental","genre_codex":"empirical","genre_gemma":"empirical","genre_scores_codex":[0.9929259,0.003448973,0.00122185,0.00008732173,0.00002060379,0.00002711426,0.0005612658,0.0000913516,0.001615693],"genre_scores_gemma":[0.9958617,0.001716954,0.001145489,0.00004461122,0.000005634685,0.00001299623,0.0003278719,0.000009314742,0.0008755512],"genre_candidate":"empirical","genre_consensus":"empirical","teacher_disagreement_score":0.001197109,"threshold_uncertainty_score":0.003625333,"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."}}