{"id":"W1985046941","doi":"10.1371/journal.pgen.1001118","title":"Mice Doubly-Deficient in Lysosomal Hexosaminidase A and Neuraminidase 4 Show Epileptic Crises and Rapid Neuronal Loss","year":2010,"lang":"en","type":"article","venue":"PLoS Genetics","topic":"Lysosomal Storage Disorders Research","field":"Medicine","cited_by":43,"is_retracted":false,"has_abstract":true,"ca_institutions":"Montreal Neurological Institute and Hospital; McGill University; Centre Hospitalier Universitaire Sainte-Justine; University of Calgary; Université de Montréal","funders":"Kyowa Hakko Kirin; Institut National de la Santé et de la Recherche Médicale","keywords":"HEXA; Tay-Sachs disease; Ganglioside; Sandhoff disease; Biology; Hexosaminidase; Lysosomal storage disease; Knockout mouse; Sialidase; Endocrinology; Internal medicine; Immunology; Genetics; Neuraminidase; Gene; Medicine; Disease; Biochemistry","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":[0.0001760379,0.001321199,0.0006025565,0.001119701,0.0003020957,0.0004351751,0.00053417,0.001002747,0.003178207],"category_scores_gemma":[0.0002347803,0.0004995374,0.0005215026,0.00041468,0.0006815383,0.0003630574,0.0005986579,0.001030848,0.001689227],"about_ca_system_candidate":false,"about_ca_system_consensus":false,"about_ca_system_score_codex":0.0003575422,"about_ca_system_score_gemma":0.0002647823,"about_ca_topic_candidate":false,"about_ca_topic_consensus":false,"about_ca_topic_score_codex":0.0007998964,"about_ca_topic_score_gemma":0.001237362,"domain_scores_codex":[0.9996369,0.00002868011,0.00005001299,0.0001084717,0.00009959826,0.00007630407],"domain_scores_gemma":[0.9993814,0.00005952476,0.0002792985,0.00004656782,0.00003327078,0.0001999898],"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":[0.0002036457,0.00003773348,0.0003804087,0.00003355534,0.00002229394,0.0003917929,0.00003572963,0.00007851007,0.9971976,0.0002074144,0.000115543,0.00129561],"study_design_scores_gemma":[0.0001710058,0.0007701918,0.02016198,0.00004161708,0.0001365939,0.006934722,0.0001826166,0.001638124,0.9629278,0.0004998135,0.006482997,0.0000525251],"study_design_candidate":"bench_or_experimental","study_design_consensus":"bench_or_experimental","genre_codex":"empirical","genre_gemma":"empirical","genre_scores_codex":[0.9889598,0.001459451,0.00422574,0.0001910656,0.00009670772,0.00004719032,0.002287734,0.0007478858,0.001984452],"genre_scores_gemma":[0.9703743,0.002175604,0.006769037,0.000150017,0.00004714546,0.0001707827,0.003159335,0.0002779979,0.01687577],"genre_candidate":"empirical","genre_consensus":"empirical","teacher_disagreement_score":0.003178207,"threshold_uncertainty_score":0.01063216,"prediction_status":"machine_predicted_unvalidated"},"machine_scores":{"provisional":true,"baseline":true,"maturity_gate_passed":false,"score_opus":0.03236438476171213,"score_gpt":0.2871552903635898,"score_spread":0.2547909056018777,"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."}}