{"id":"W4414710100","doi":"10.1016/j.omtn.2025.102728","title":"Targeting AASS alleviates neurotoxicity and improves mitochondrial function in astrocyte models for pyridoxine-dependent epilepsy","year":2025,"lang":"en","type":"article","venue":"Molecular Therapy — Nucleic Acids","topic":"Metabolism and Genetic Disorders","field":"Biochemistry, Genetics and Molecular Biology","cited_by":3,"is_retracted":false,"has_abstract":true,"ca_institutions":"University of British Columbia","funders":"ZonMw","keywords":"Neurotoxicity; Catabolism; Epilepsy; Mitochondrion; Oxidative phosphorylation; Reactive oxygen species; Astrocyte; Induced pluripotent stem cell; Oxidative stress","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":[0.0001781092,0.0004763546,0.0004000285,0.0003509269,0.0002547066,0.0003120429,0.0002933085,0.0004092168,0.001484281],"category_scores_gemma":[0.0001117559,0.0001106092,0.0003536452,0.0002131429,0.0003122358,0.0003134108,0.0001739904,0.0007754443,0.0002868951],"about_ca_system_candidate":false,"about_ca_system_consensus":false,"about_ca_system_score_codex":0.0003037201,"about_ca_system_score_gemma":0.0004585032,"about_ca_topic_candidate":false,"about_ca_topic_consensus":false,"about_ca_topic_score_codex":0.001973557,"about_ca_topic_score_gemma":0.006951592,"domain_scores_codex":[0.9999088,0.00001581578,0.0000112543,0.000012717,0.00002665394,0.00002471046],"domain_scores_gemma":[0.9999427,0.000003991775,0.00001665719,0.00000832289,0.00001048829,0.00001778975],"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":[0.0007398333,0.0001863507,0.0001462934,0.000113392,0.00003314997,0.00009579255,0.00002308574,0.0001697517,0.991585,0.0002600507,0.0002551291,0.0063922],"study_design_scores_gemma":[0.0001938626,0.002510885,0.003230326,0.00002684097,0.0000897936,0.0003715483,0.00009210991,0.001572325,0.9844929,0.0004270365,0.00697647,0.000015958],"study_design_candidate":"bench_or_experimental","study_design_consensus":"bench_or_experimental","genre_codex":"empirical","genre_gemma":"empirical","genre_scores_codex":[0.9785281,0.004576756,0.01118519,0.0006855464,0.0002862372,0.0001876262,0.0007868329,0.0004995108,0.003264156],"genre_scores_gemma":[0.9877267,0.003609699,0.003358072,0.00009420971,0.000019698,0.0000886418,0.0006560379,0.00003535301,0.004411587],"genre_candidate":"empirical","genre_consensus":"empirical","teacher_disagreement_score":0.001973557,"threshold_uncertainty_score":0.004965365,"prediction_status":"machine_predicted_unvalidated"},"machine_scores":{"provisional":true,"baseline":true,"maturity_gate_passed":false,"score_opus":0.006588864687119145,"score_gpt":0.231653587115678,"score_spread":0.2250647224285589,"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."}}