{"id":"W3189405174","doi":"10.15252/embr.202051991","title":"The biochemical basis of mitochondrial dysfunction in Zellweger Spectrum Disorder","year":2021,"lang":"en","type":"article","venue":"EMBO Reports","topic":"Peroxisome Proliferator-Activated Receptors","field":"Biochemistry, Genetics and Molecular Biology","cited_by":58,"is_retracted":false,"has_abstract":false,"ca_institutions":"McGill University Health Centre; McGill University; McGill Genome Centre","funders":"National Institute of General Medical Sciences; National Institute of Diabetes and Digestive and Kidney Diseases; NIH Office of the Director; Canadian Institutes of Health Research; National Cancer Institute; National Institutes of Health; Howard Hughes Medical Institute; Nora Eccles Treadwell Foundation; Deutsche Forschungsgemeinschaft; Global Foundation for Peroxisomal Disorders; Jane Coffin Childs Memorial Fund for Medical Research","keywords":"Peroxisome; Biology; Zellweger syndrome; Mitochondrion; Biogenesis; Mitochondrial biogenesis; Cell biology; Phenotype; Peroxisomal disorder; Mitochondrial DNA; Mitophagy; Gene; Mitochondrial disease; Genetics; Autophagy","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":[0.0001701723,0.0004798417,0.0002630968,0.001044869,0.0003664035,0.0003647324,0.0004428747,0.0008509629,0.001034764],"category_scores_gemma":[0.000465055,0.0001740577,0.0001426814,0.0004235231,0.0004933665,0.0004422296,0.0003651532,0.0005439151,0.0002652872],"about_ca_system_candidate":false,"about_ca_system_consensus":false,"about_ca_system_score_codex":0.0004957474,"about_ca_system_score_gemma":0.0002605655,"about_ca_topic_candidate":false,"about_ca_topic_consensus":false,"about_ca_topic_score_codex":0.0006018514,"about_ca_topic_score_gemma":0.0003782263,"domain_scores_codex":[0.9999208,0.00001540062,0.00001617159,0.00001331591,0.00002003843,0.00001425449],"domain_scores_gemma":[0.999849,0.00004169445,0.00004655608,0.00001179906,0.00001649791,0.00003445349],"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":[0.003088931,0.0001838833,0.04743188,0.0005890417,0.0001635741,0.1085351,0.000561955,0.002133806,0.7830423,0.01944329,0.002015537,0.03281069],"study_design_scores_gemma":[0.000240616,0.0007398446,0.1959522,0.0001845825,0.0003976065,0.2206203,0.001438216,0.005777925,0.508964,0.04142435,0.02414757,0.0001128572],"study_design_candidate":"bench_or_experimental","study_design_consensus":"bench_or_experimental","genre_codex":"empirical","genre_gemma":"empirical","genre_scores_codex":[0.9680564,0.02056347,0.003028203,0.002868345,0.0001899209,0.00003639296,0.0006366351,0.000131359,0.004489273],"genre_scores_gemma":[0.9951696,0.003391919,0.0007066809,0.000106949,0.0000498025,0.000008990104,0.0001534405,0.000007187907,0.000405462],"genre_candidate":"empirical","genre_consensus":"empirical","teacher_disagreement_score":0.001044869,"threshold_uncertainty_score":0.003596902,"prediction_status":"machine_predicted_unvalidated"},"machine_scores":{"provisional":true,"baseline":true,"maturity_gate_passed":false,"score_opus":0.004528407044562067,"score_gpt":0.2203226855621931,"score_spread":0.215794278517631,"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."}}