{"id":"W2058131268","doi":"10.2353/ajpath.2009.081096","title":"Increased Activity and Altered Subcellular Distribution of Lysosomal Enzymes Determine Neuronal Vulnerability in Niemann-Pick Type C1-Deficient Mice","year":2009,"lang":"en","type":"article","venue":"American Journal Of Pathology","topic":"Lysosomal Storage Disorders Research","field":"Medicine","cited_by":89,"is_retracted":false,"has_abstract":false,"ca_institutions":"University of Alberta","funders":"Natural Sciences and Engineering Research Council of Canada; Canadian Institutes of Health Research; Alberta Heritage Foundation for Medical Research; Fondation pour la Recherche Médicale","keywords":"Vulnerability (computing); Enzyme; Lysosome; Neuroscience; Biology; Distribution (mathematics); Niemann–Pick disease; Cell biology; Chemistry; Biochemistry; Computer science; Cholesterol","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":[0.0003570396,0.001663584,0.0009012527,0.003384105,0.0007439445,0.001010278,0.0007710173,0.001718105,0.003270558],"category_scores_gemma":[0.000590097,0.0009788428,0.0006098627,0.0008584772,0.001349531,0.0008194626,0.0006214464,0.002110497,0.0008785663],"about_ca_system_candidate":false,"about_ca_system_consensus":false,"about_ca_system_score_codex":0.001123638,"about_ca_system_score_gemma":0.0005872818,"about_ca_topic_candidate":false,"about_ca_topic_consensus":false,"about_ca_topic_score_codex":0.002395139,"about_ca_topic_score_gemma":0.003792513,"domain_scores_codex":[0.9994773,0.00004572319,0.00007369794,0.0001479585,0.0001254718,0.0001297437],"domain_scores_gemma":[0.9988318,0.0001502702,0.000496715,0.00006667373,0.00007025205,0.0003842297],"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":[0.00116899,0.0001380457,0.0007129214,0.00007668447,0.00003391591,0.0006532894,0.00009113031,0.0002571577,0.9950358,0.0004065212,0.0001265505,0.001299021],"study_design_scores_gemma":[0.0002264247,0.0007363263,0.03127575,0.00006873302,0.0002171335,0.005725486,0.0003262299,0.002702406,0.9556188,0.0009464081,0.002065688,0.00009061604],"study_design_candidate":"bench_or_experimental","study_design_consensus":"bench_or_experimental","genre_codex":"empirical","genre_gemma":"empirical","genre_scores_codex":[0.9952212,0.0009725344,0.001226373,0.0001828781,0.00003710579,0.00003108251,0.001333114,0.0001514209,0.0008441818],"genre_scores_gemma":[0.9913078,0.001309575,0.002412711,0.00008606635,0.0000212242,0.00007607522,0.0008416493,0.0001680136,0.003776848],"genre_candidate":"empirical","genre_consensus":"empirical","teacher_disagreement_score":0.003384105,"threshold_uncertainty_score":0.01094115,"prediction_status":"machine_predicted_unvalidated"},"machine_scores":{"provisional":true,"baseline":true,"maturity_gate_passed":false,"score_opus":0.01748653757516655,"score_gpt":0.3000471163895236,"score_spread":0.2825605788143571,"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."}}