{"id":"W2912077551","doi":"10.1002/jimd.12047","title":"Argininosuccinic aciduria: Recent pathophysiological insights and therapeutic prospects","year":2019,"lang":"en","type":"review","venue":"Journal of Inherited Metabolic Disease","topic":"Metabolism and Genetic Disorders","field":"Biochemistry, Genetics and Molecular Biology","cited_by":49,"is_retracted":false,"has_abstract":true,"ca_institutions":"","funders":"H2020 European Research Council; Medical Research Council; Spendenstiftung Bank Vontobel; Israel Science Foundation; Medical Research Council Canada; Schweizerischer Nationalfonds zur Förderung der Wissenschaftlichen Forschung; National Institute for Health and Care Research; Action Medical Research","keywords":"Medicine; Argininosuccinate lyase; Urea cycle; Newborn screening; Disease; Intensive care medicine; Pathophysiology; Bioinformatics; Clinical trial; Pediatrics; Arginine; Internal medicine; Biology","routes":{"ca_aff":false,"ca_fund":true,"ca_venue":false,"about_ca":false,"invisible_to_affiliation_only":true},"retraction":null,"screen":null,"direct_labels":[],"prediction":{"model_version":"metacan-v3-hybrid-931329e0061c","candidate_categories":[],"consensus_categories":[],"category_scores_codex":[0.001102917,0.0006127797,0.0007819977,0.001214679,0.0001891946,0.00107314,0.000541546,0.0009929906,0.001176303],"category_scores_gemma":[0.0009902337,0.0001883251,0.0004064526,0.001063291,0.0006614859,0.001351369,0.0006834792,0.001629663,0.0004914508],"about_ca_system_candidate":false,"about_ca_system_consensus":false,"about_ca_system_score_codex":0.0005514047,"about_ca_system_score_gemma":0.0007929769,"about_ca_topic_candidate":false,"about_ca_topic_consensus":false,"about_ca_topic_score_codex":0.0003205577,"about_ca_topic_score_gemma":0.0005338007,"domain_scores_codex":[0.9997693,0.00005469689,0.0000454059,0.00005540808,0.0000481452,0.00002712365],"domain_scores_gemma":[0.9994674,0.0002340195,0.00007689624,0.0000211602,0.0001300428,0.00007058745],"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":[0.000586649,0.0001746288,0.001246056,0.01351909,0.0002026455,0.001196013,0.0001765866,0.0007324662,0.009861507,0.01208525,0.01776126,0.9424579],"study_design_scores_gemma":[0.0001128585,0.0006711051,0.002583981,0.002790291,0.0004793739,0.007433138,0.0002108643,0.0008565641,0.006475064,0.01369484,0.9646122,0.00007970601],"study_design_candidate":"not_applicable","study_design_consensus":null,"genre_codex":"review","genre_gemma":"review","genre_scores_codex":[0.0005311618,0.9973996,0.0003685603,0.0009425108,0.0002100578,0.000002575067,0.00001083205,0.000009003395,0.0005256144],"genre_scores_gemma":[0.005469412,0.992125,0.0006676895,0.0005674838,0.0008178843,0.000008835352,0.00004585485,0.000004492372,0.0002933212],"genre_candidate":"review","genre_consensus":"review","teacher_disagreement_score":0.001214679,"threshold_uncertainty_score":0.005832911,"prediction_status":"machine_predicted_unvalidated"},"machine_scores":{"provisional":true,"baseline":true,"maturity_gate_passed":false,"score_opus":0.03210083483663462,"score_gpt":0.30850972555776,"score_spread":0.2764088907211254,"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."}}