{"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":"metacan-v3-hybrid-931329e0061c","candidate_categories":[],"consensus_categories":[],"category_scores_codex":[0.0008803621,0.0007362924,0.001749178,0.00145901,0.0002319574,0.001355187,0.0008561021,0.00131517,0.004304613],"category_scores_gemma":[0.001055163,0.0002393665,0.0005927221,0.001303902,0.0005133863,0.001547319,0.0007943999,0.002225097,0.001593308],"about_ca_system_candidate":false,"about_ca_system_consensus":false,"about_ca_system_score_codex":0.0003647121,"about_ca_system_score_gemma":0.001012717,"about_ca_topic_candidate":false,"about_ca_topic_consensus":false,"about_ca_topic_score_codex":0.0004774381,"about_ca_topic_score_gemma":0.001256978,"domain_scores_codex":[0.9998087,0.00003384882,0.00003430084,0.00003514555,0.00006312464,0.00002483279],"domain_scores_gemma":[0.9994807,0.000289027,0.00006926983,0.00001242664,0.0001048155,0.00004388993],"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":[0.0002368621,0.0001333644,0.0002392324,0.01910666,0.0001525767,0.0002640227,0.00005861251,0.0003344842,0.002476067,0.003936511,0.03336567,0.939696],"study_design_scores_gemma":[0.00007154047,0.0002007413,0.0005376159,0.00416819,0.0002949651,0.001162175,0.00006638173,0.0001574205,0.0007274248,0.00262185,0.9899665,0.0000250887],"study_design_candidate":"not_applicable","study_design_consensus":null,"genre_codex":"review","genre_gemma":"review","genre_scores_codex":[0.00009672013,0.9988474,0.0001254061,0.0002820105,0.00016336,0.000003370363,0.00001706162,0.000006894004,0.0004578091],"genre_scores_gemma":[0.0007412111,0.9983133,0.0002128264,0.0002440116,0.0001834415,0.000005460263,0.00002553154,0.000001436561,0.0002727783],"genre_candidate":"review","genre_consensus":"review","teacher_disagreement_score":0.004304613,"threshold_uncertainty_score":0.01440036,"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."}}