{"id":"W4360613774","doi":"10.1038/s41598-023-31777-4","title":"Optimizing human α-galactosidase for treatment of Fabry disease","year":2023,"lang":"en","type":"article","venue":"Scientific Reports","topic":"Lysosomal Storage Disorders Research","field":"Medicine","cited_by":18,"is_retracted":false,"has_abstract":true,"ca_institutions":"Emergent BioSolutions (Canada)","funders":"University of Pittsburgh","keywords":"Globotriaosylceramide; Fabry disease; Lysosomal storage disease; Kidney; Enzyme; Biology; Enzyme replacement therapy; Biochemistry; Molecular biology; Chemistry; Medicine; Disease; Internal medicine; Endocrinology","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":[0.0002331994,0.0003492568,0.0002083601,0.0001794065,0.00007350129,0.0003537531,0.0001909537,0.0002543418,0.0005836363],"category_scores_gemma":[0.0001574273,0.00009118245,0.0002613139,0.0001961396,0.0001370953,0.0001255543,0.0001860683,0.0004155764,0.0002600652],"about_ca_system_candidate":false,"about_ca_system_consensus":false,"about_ca_system_score_codex":0.0003719174,"about_ca_system_score_gemma":0.0001498958,"about_ca_topic_candidate":false,"about_ca_topic_consensus":false,"about_ca_topic_score_codex":0.0005810928,"about_ca_topic_score_gemma":0.0007187887,"domain_scores_codex":[0.9998639,0.00002928206,0.00001258242,0.00002630478,0.00004537327,0.00002248495],"domain_scores_gemma":[0.9999559,0.000006555328,0.00001611726,0.000004439128,0.000009384908,0.000007590575],"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":[0.00009663228,0.00004835346,0.0002369641,0.0000277244,0.00001219309,0.00003966825,0.000009557378,0.0006487421,0.9944772,0.00008983374,0.00006684031,0.004246276],"study_design_scores_gemma":[0.00002138592,0.0004035486,0.0007600228,0.000005599552,0.00002088203,0.0001789487,0.000007807153,0.001348777,0.9941987,0.00003508305,0.003014144,0.000005181234],"study_design_candidate":"bench_or_experimental","study_design_consensus":"bench_or_experimental","genre_codex":"empirical","genre_gemma":"empirical","genre_scores_codex":[0.9787928,0.003435672,0.01563585,0.0001678095,0.00003768849,0.0000839912,0.0003303838,0.0001192428,0.001396615],"genre_scores_gemma":[0.983138,0.001663591,0.01308056,0.00008791242,0.000006213556,0.0000313214,0.0004876784,0.00003998369,0.001464727],"genre_candidate":"empirical","genre_consensus":"empirical","teacher_disagreement_score":0.0005836363,"threshold_uncertainty_score":0.002698481,"prediction_status":"machine_predicted_unvalidated"},"machine_scores":{"provisional":true,"baseline":true,"maturity_gate_passed":false,"score_opus":0.06192194712630553,"score_gpt":0.3642670494778133,"score_spread":0.3023451023515078,"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."}}