{"id":"W2900616353","doi":"10.1016/j.ymgme.2018.11.011","title":"Enzyme enhancement therapeutics for lysosomal storage diseases: Current status and perspective","year":2018,"lang":"en","type":"review","venue":"Molecular Genetics and Metabolism","topic":"Lysosomal Storage Disorders Research","field":"Medicine","cited_by":26,"is_retracted":false,"has_abstract":false,"ca_institutions":"Simon Fraser University","funders":"Michael Smith Health Research BC","keywords":"Endoplasmic-reticulum-associated protein degradation; Proteostasis; Lysosome; Endoplasmic reticulum; Lysosomal storage disease; Cell biology; Enzyme replacement therapy; Chemical chaperone; Biology; Enzyme; Glucocerebrosidase; Biochemistry; Unfolded protein response; Medicine; Disease","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":"codex-gemma-dda1882f352a","candidate_categories":["metaepi_narrow"],"consensus_categories":[],"category_scores_codex":[0.0001627431,0.0005871311,0.001499701,0.0002580834,0.0001467493,0.0001088304,0.0001609488,0.0002374146,0.00002829316],"category_scores_gemma":[0.00008439284,0.0004708283,0.0004248867,0.0002163672,0.0002868483,0.0000257749,0.0002510772,0.0003322408,0.000008008122],"about_ca_system_candidate":false,"about_ca_system_consensus":false,"about_ca_system_score_codex":0.0001406357,"about_ca_system_score_gemma":0.0006013095,"about_ca_topic_candidate":false,"about_ca_topic_consensus":false,"about_ca_topic_score_codex":0.00001431262,"about_ca_topic_score_gemma":0.000002187823,"domain_scores_codex":[0.9972913,0.0001595102,0.0004498746,0.0008801757,0.0005249089,0.0006942528],"domain_scores_gemma":[0.9982694,0.00006216959,0.0001898984,0.0005429859,0.0004235192,0.0005120316],"domain_codex":null,"domain_gemma":null,"domain_candidate":null,"domain_consensus":null,"study_design_codex":"design_other","study_design_gemma":"not_applicable","study_design_scores_codex":[0.00006187506,0.0003192564,0.00000884305,0.007778276,0.001063303,0.00002929287,0.000373581,1.582962e-7,0.0001064013,0.001485374,0.0001648714,0.9886088],"study_design_scores_gemma":[0.001359744,0.0003238312,0.00009113001,0.00105945,0.005148891,0.00002052133,0.00008448636,0.00005798158,0.00007981498,0.0005146363,0.9908041,0.000455393],"study_design_candidate":"not_applicable","study_design_consensus":null,"genre_codex":"review","genre_gemma":"review","genre_scores_codex":[0.001125765,0.9918302,0.003156573,0.00005214204,0.0003418613,0.003071753,0.000297366,0.00002300816,0.0001013116],"genre_scores_gemma":[0.0003798656,0.9971745,0.0009867667,0.0001267384,0.0003979248,0.0004361809,0.0001993795,0.0001319393,0.0001666903],"genre_candidate":"review","genre_consensus":"review","teacher_disagreement_score":0.9906393,"threshold_uncertainty_score":0.9997743,"prediction_status":"machine_predicted_unvalidated"},"machine_scores":{"provisional":true,"baseline":true,"maturity_gate_passed":false,"score_opus":0.04553291050281619,"score_gpt":0.3809003642360579,"score_spread":0.3353674537332417,"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."}}