{"id":"W4286001490","doi":"10.21203/rs.3.rs-1839772/v1","title":"Riluzole partially restores RNA polymerase III complex assembly in cells expressing the leukodystrophy-causative variant POLR3B R103H","year":2022,"lang":"en","type":"preprint","venue":"Research Square","topic":"RNA regulation and disease","field":"Biochemistry, Genetics and Molecular Biology","cited_by":0,"is_retracted":false,"has_abstract":false,"ca_institutions":"McGill University; Montreal Clinical Research Institute","funders":"Canadian Institutes of Health Research; Fonds de Recherche du Québec - Santé; Compute Canada","keywords":"Riluzole; Leukodystrophy; Polymerase; Cell biology; Chemistry; RNA polymerase; Biology; Genetics; Molecular biology; RNA; Medicine; Glutamate receptor; DNA; Pathology; Gene; Disease","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":["metaepi_narrow"],"consensus_categories":[],"category_scores_codex":[0.001336971,0.0003594539,0.0003448228,0.0002754332,0.0006140905,0.0002836772,0.0009556525,0.0002979465,0.0004711769],"category_scores_gemma":[0.000253619,0.0003028199,0.0002572577,0.0003042989,0.000360438,0.000009905196,0.002511686,0.001143378,0.00005003577],"about_ca_system_candidate":false,"about_ca_system_consensus":false,"about_ca_system_score_codex":0.0002084096,"about_ca_system_score_gemma":0.001244364,"about_ca_topic_candidate":false,"about_ca_topic_consensus":false,"about_ca_topic_score_codex":0.001355602,"about_ca_topic_score_gemma":0.0002790363,"domain_scores_codex":[0.9945816,0.002011224,0.0005045342,0.0009565229,0.001145236,0.0008009148],"domain_scores_gemma":[0.9977295,0.0001507604,0.00020472,0.001344251,0.0002603113,0.0003104269],"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":[0.001393605,0.000549104,0.001760442,0.0002781824,0.0001683464,0.0003900998,0.0008533196,0.01901608,0.9430628,0.0006876699,0.03001044,0.001829931],"study_design_scores_gemma":[0.01109503,0.002378394,0.2661312,0.001446303,0.0002013696,0.00005480311,0.01406636,0.03096691,0.3919538,0.003139063,0.2743387,0.004228089],"study_design_candidate":"bench_or_experimental","study_design_consensus":"bench_or_experimental","genre_codex":"empirical","genre_gemma":"empirical","genre_scores_codex":[0.9864898,0.003064347,0.0004918897,0.001823263,0.000354259,0.001580643,0.0006700667,0.00003781253,0.00548793],"genre_scores_gemma":[0.9956671,0.0003021143,0.0001804261,0.0001917144,0.0005493382,0.0004061362,0.0009712134,0.00006393372,0.001668031],"genre_candidate":"empirical","genre_consensus":"empirical","teacher_disagreement_score":0.551109,"threshold_uncertainty_score":0.9999424,"prediction_status":"machine_predicted_unvalidated"},"machine_scores":{"provisional":true,"baseline":true,"maturity_gate_passed":false,"score_opus":0.06526398616617361,"score_gpt":0.3795920408543109,"score_spread":0.3143280546881372,"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."}}