{"id":"W3004218697","doi":"10.3390/jcm9020396","title":"Brain Pathology in Mucopolysaccharidoses (MPS) Patients with Neurological Forms","year":2020,"lang":"en","type":"article","venue":"Journal of Clinical Medicine","topic":"Lysosomal Storage Disorders Research","field":"Medicine","cited_by":67,"is_retracted":false,"has_abstract":true,"ca_institutions":"Centre Hospitalier Universitaire Sainte-Justine","funders":"Canadian Institutes of Health Research; Carol Ann Foundation; Fundação de Amparo à Pesquisa do Estado de São Paulo; Royal Society of Medicine; Bennett Foundation; Wellcome Trust; Jacob Randall Foundation for Morquio","keywords":"Keratan sulfate; Medicine; Glycosaminoglycan; Pathology; Dermatan sulfate; Astrogliosis; Heparan sulfate; Mucopolysaccharidosis I; Neuroinflammation; Sulfatase; Chondroitin sulfate; Internal medicine; Biochemistry; Central nervous system; Biology; Enzyme; Anatomy; Enzyme replacement therapy","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":[0.0001390272,0.0005764703,0.0002740099,0.00104602,0.000486825,0.0002595774,0.0001394201,0.0002937551,0.001282333],"category_scores_gemma":[0.0003354394,0.0002041463,0.0001514452,0.000336921,0.0003565627,0.0002306699,0.0003120879,0.0002252612,0.0002004843],"about_ca_system_candidate":false,"about_ca_system_consensus":false,"about_ca_system_score_codex":0.0001694652,"about_ca_system_score_gemma":0.0001863741,"about_ca_topic_candidate":false,"about_ca_topic_consensus":false,"about_ca_topic_score_codex":0.0009703674,"about_ca_topic_score_gemma":0.001354137,"domain_scores_codex":[0.9999015,0.00001595913,0.00001837553,0.0000302744,0.00001883657,0.00001509235],"domain_scores_gemma":[0.9998733,0.00001395473,0.00005736173,0.000007141311,0.00001604252,0.00003218751],"domain_codex":null,"domain_gemma":null,"domain_candidate":null,"domain_consensus":null,"study_design_codex":"observational","study_design_gemma":"observational","study_design_scores_codex":[0.002520424,0.0002686832,0.7399735,0.0002128407,0.0002794733,0.03483088,0.001040202,0.0001712302,0.1999958,0.0001320847,0.0009329529,0.0196419],"study_design_scores_gemma":[0.0000381376,0.0005002083,0.9545662,0.00002188008,0.00008562813,0.03837294,0.0003702373,0.0001044871,0.005228817,0.0001299311,0.0005701401,0.00001134388],"study_design_candidate":"observational","study_design_consensus":"observational","genre_codex":"empirical","genre_gemma":"empirical","genre_scores_codex":[0.9990695,0.0003481152,0.0000935719,0.00002204372,0.000003068002,0.000008636124,0.0001162954,0.00001003109,0.000328694],"genre_scores_gemma":[0.9990816,0.0002943074,0.0002224874,0.00002715851,0.000008773903,0.000008905216,0.000187713,0.000002731174,0.0001662193],"genre_candidate":"empirical","genre_consensus":"empirical","teacher_disagreement_score":0.001282333,"threshold_uncertainty_score":0.004289806,"prediction_status":"machine_predicted_unvalidated"},"machine_scores":{"provisional":true,"baseline":true,"maturity_gate_passed":false,"score_opus":0.08093144261731172,"score_gpt":0.4113539713574659,"score_spread":0.3304225287401542,"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."}}