{"id":"W1533651529","doi":"10.1111/j.1742-4658.2010.07588.x","title":"The pharmacological chaperone isofagomine increases the activity of the Gaucher disease L444P mutant form of <b>β</b>‐glucosidase","year":2010,"lang":"en","type":"article","venue":"FEBS Journal","topic":"Lysosomal Storage Disorders Research","field":"Medicine","cited_by":158,"is_retracted":false,"has_abstract":true,"ca_institutions":"Hospital for Sick Children","funders":"National Institutes of Health","keywords":"Endogeny; Incubation; Enzyme; Biology; Pharmacology; Biochemistry","routes":{"ca_aff":true,"ca_fund":false,"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":[0.00009995385,0.0003175814,0.0003125467,0.0001868242,0.0001052946,0.0002899203,0.0001702566,0.0003428351,0.0009815028],"category_scores_gemma":[0.0001250253,0.0001330952,0.0002366477,0.0001213862,0.0001695607,0.0001426979,0.000190503,0.0005243044,0.000338045],"about_ca_system_candidate":false,"about_ca_system_consensus":false,"about_ca_system_score_codex":0.0003251763,"about_ca_system_score_gemma":0.0001979092,"about_ca_topic_candidate":false,"about_ca_topic_consensus":false,"about_ca_topic_score_codex":0.0007155853,"about_ca_topic_score_gemma":0.00107157,"domain_scores_codex":[0.9998643,0.00003304341,0.00001240463,0.00002096793,0.00003150845,0.00003771848],"domain_scores_gemma":[0.9999113,0.00001261203,0.00002705803,0.000009232839,0.000009613285,0.00003017344],"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":[0.000133206,0.0000287679,0.00008402096,0.00001192468,0.000003842175,0.00002138787,0.000003674938,0.00001176953,0.9991641,0.00001111366,0.00002891848,0.000497343],"study_design_scores_gemma":[0.00003094339,0.0006660598,0.003404881,0.000003482533,0.0000146421,0.0001713095,0.00001560782,0.0003063683,0.9942871,0.00001211958,0.001084187,0.000003330194],"study_design_candidate":"bench_or_experimental","study_design_consensus":"bench_or_experimental","genre_codex":"empirical","genre_gemma":"empirical","genre_scores_codex":[0.9979626,0.0006139842,0.000460558,0.00009808016,0.00001697749,0.00001517965,0.0001464557,0.00009338225,0.0005927578],"genre_scores_gemma":[0.9964319,0.0005173893,0.001012682,0.0000921953,0.000009714478,0.00002888576,0.0004013044,0.0000232004,0.001482682],"genre_candidate":"empirical","genre_consensus":"empirical","teacher_disagreement_score":0.0009815028,"threshold_uncertainty_score":0.003283501,"prediction_status":"machine_predicted_unvalidated"},"machine_scores":{"provisional":true,"baseline":true,"maturity_gate_passed":false,"score_opus":0.02378432022280621,"score_gpt":0.3209401809721473,"score_spread":0.2971558607493411,"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."}}