{"id":"W4398171854","doi":"10.3390/cells13100877","title":"Heterologous HSPC Transplantation Rescues Neuroinflammation and Ameliorates Peripheral Manifestations in the Mouse Model of Lysosomal Transmembrane Enzyme Deficiency, MPS IIIC","year":2024,"lang":"en","type":"article","venue":"Cells","topic":"Lysosomal Storage Disorders Research","field":"Medicine","cited_by":4,"is_retracted":false,"has_abstract":true,"ca_institutions":"McGill University; Université de Montréal; Centre Hospitalier Universitaire Sainte-Justine","funders":"Canadian Glycomics Network; Canadian Institutes of Health Research; Kyowa Hakko Kirin; Sanfilippo Children's Foundation","keywords":"Neuroinflammation; Microglia; Heparan sulfate; Lysosomal storage disease; Enzyme replacement therapy; Transplantation; Astrogliosis; Biology; Cell biology; Immunology; Pathology; Medicine; Neuroscience; Internal medicine; Central nervous system; Biochemistry; Inflammation; Disease; Glycosaminoglycan","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":[0.0001714487,0.0008730785,0.0006829889,0.0007228395,0.0002513524,0.0003794504,0.0003114131,0.0006530972,0.0009696872],"category_scores_gemma":[0.0001418242,0.0002236018,0.0004700065,0.0002535675,0.0003881869,0.0002579508,0.0002241326,0.001317974,0.0003765198],"about_ca_system_candidate":false,"about_ca_system_consensus":false,"about_ca_system_score_codex":0.0003444107,"about_ca_system_score_gemma":0.0004184222,"about_ca_topic_candidate":false,"about_ca_topic_consensus":false,"about_ca_topic_score_codex":0.001419744,"about_ca_topic_score_gemma":0.001993655,"domain_scores_codex":[0.9998292,0.00001720713,0.0000228443,0.00003590282,0.00004241099,0.00005241785],"domain_scores_gemma":[0.9997403,0.00001139927,0.00008895791,0.00002455882,0.00002622664,0.0001086351],"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":[0.0004110651,0.0001806853,0.000160116,0.00009340839,0.00001483636,0.0002228603,0.00003056847,0.0002059304,0.9965868,0.0001048598,0.0001581293,0.001830772],"study_design_scores_gemma":[0.000195624,0.004050818,0.01105175,0.0001025596,0.000122633,0.001109281,0.0001675859,0.002438211,0.976146,0.000233461,0.004354584,0.00002757958],"study_design_candidate":"bench_or_experimental","study_design_consensus":"bench_or_experimental","genre_codex":"empirical","genre_gemma":"empirical","genre_scores_codex":[0.9938911,0.001468613,0.001709351,0.0002000343,0.00009594064,0.00005867967,0.001167772,0.0003989407,0.001009533],"genre_scores_gemma":[0.9894432,0.002046967,0.002067518,0.00014041,0.0000328602,0.0001091487,0.001152506,0.00009382736,0.00491346],"genre_candidate":"empirical","genre_consensus":"empirical","teacher_disagreement_score":0.001419744,"threshold_uncertainty_score":0.003243923,"prediction_status":"machine_predicted_unvalidated"},"machine_scores":{"provisional":true,"baseline":true,"maturity_gate_passed":false,"score_opus":0.02335333393713621,"score_gpt":0.2769768670176262,"score_spread":0.25362353308049,"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."}}