{"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":"codex-gemma-dda1882f352a","candidate_categories":[],"consensus_categories":[],"category_scores_codex":[0.0001352367,0.0001221773,0.0001452563,0.00002866556,0.00005453424,0.00001759048,0.00007149552,0.0001752322,0.00007272154],"category_scores_gemma":[0.0003306706,0.00009635313,0.0001022616,0.0001893845,0.00007808633,0.000003706831,0.00009574213,0.0001207088,0.000002908182],"about_ca_system_candidate":false,"about_ca_system_consensus":false,"about_ca_system_score_codex":0.00002175499,"about_ca_system_score_gemma":0.0001538478,"about_ca_topic_candidate":false,"about_ca_topic_consensus":false,"about_ca_topic_score_codex":0.00002696533,"about_ca_topic_score_gemma":0.00006971017,"domain_scores_codex":[0.9988356,0.00006482239,0.0003641198,0.0003588358,0.0001638861,0.0002127301],"domain_scores_gemma":[0.9992648,0.00001639654,0.0001417875,0.0004565876,0.00007105771,0.00004937542],"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":[0.00006447384,0.00009011646,0.02299448,0.000007440942,0.00003962463,0.00003884158,0.00001663407,0.00002014847,0.9724867,0.00001177823,0.003680917,0.0005487839],"study_design_scores_gemma":[0.000242966,0.00004387445,0.001600233,0.00001027228,0.00000817647,0.0001189305,0.00005535456,0.000005631132,0.9609717,0.000134215,0.03669774,0.0001109626],"study_design_candidate":"bench_or_experimental","study_design_consensus":"bench_or_experimental","genre_codex":"empirical","genre_gemma":"empirical","genre_scores_codex":[0.9961057,0.001246453,0.0001732357,0.0004212818,0.0009591369,0.0001158137,0.000002916308,0.000008288794,0.0009671585],"genre_scores_gemma":[0.9983261,0.000199831,0.0001685007,0.00005056741,0.0003005481,0.00002357162,0.00008063621,0.00001941266,0.0008308888],"genre_candidate":"empirical","genre_consensus":"empirical","teacher_disagreement_score":0.03301682,"threshold_uncertainty_score":0.3929167,"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."}}