{"id":"W2133774695","doi":"10.1093/hmg/9.7.1075","title":"Characterization of the sialidase molecular defects in sialidosis patients suggests the structural organization of the lysosomal multienzyme complex","year":2000,"lang":"en","type":"article","venue":"Human Molecular Genetics","topic":"Lysosomal Storage Disorders Research","field":"Medicine","cited_by":81,"is_retracted":false,"has_abstract":true,"ca_institutions":"Université de Montréal; Centre Hospitalier Universitaire Sainte-Justine","funders":"Medical Research Council Canada","keywords":"Biology; Sialidase; Cell biology; Lysosome; Genetics; Biochemistry; Evolutionary biology; Enzyme; Neuraminidase","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":[0.00006107504,0.0006052883,0.000210117,0.0002841601,0.0002052162,0.00018669,0.000114967,0.0002711742,0.001707026],"category_scores_gemma":[0.0003147808,0.0001406291,0.0003177183,0.0001936063,0.0002130299,0.0001070378,0.0001456569,0.0002618686,0.000344379],"about_ca_system_candidate":false,"about_ca_system_consensus":false,"about_ca_system_score_codex":0.0001175199,"about_ca_system_score_gemma":0.0001260425,"about_ca_topic_candidate":false,"about_ca_topic_consensus":false,"about_ca_topic_score_codex":0.0007071597,"about_ca_topic_score_gemma":0.0005525239,"domain_scores_codex":[0.999941,0.00001048938,0.000006573096,0.00001737477,0.00001450474,0.00001008121],"domain_scores_gemma":[0.9999267,0.00001738387,0.00002007073,0.000006910937,0.000006322545,0.00002270717],"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":[0.0009667375,0.0002384181,0.3559899,0.00009079999,0.0001770597,0.01330201,0.0007324893,0.001711986,0.6022097,0.0005744891,0.0002964671,0.02371006],"study_design_scores_gemma":[0.0001681631,0.001209738,0.8303657,0.0000412933,0.0002568701,0.07168617,0.0005777125,0.006549979,0.08400984,0.0008496174,0.00423394,0.00005089543],"study_design_candidate":"bench_or_experimental","study_design_consensus":"bench_or_experimental","genre_codex":"empirical","genre_gemma":"empirical","genre_scores_codex":[0.999108,0.00008985948,0.0004672444,0.00001676738,0.000001399386,0.000003568874,0.00008882487,0.000008562247,0.0002157445],"genre_scores_gemma":[0.9989827,0.00009003168,0.0005948184,0.000007595043,0.00000198783,0.000004085217,0.0001562228,0.000004444775,0.0001580105],"genre_candidate":"empirical","genre_consensus":"empirical","teacher_disagreement_score":0.001707026,"threshold_uncertainty_score":0.005710542,"prediction_status":"machine_predicted_unvalidated"},"machine_scores":{"provisional":true,"baseline":true,"maturity_gate_passed":false,"score_opus":0.01259681805518243,"score_gpt":0.2612228210355955,"score_spread":0.248626002980413,"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."}}