{"id":"W2124292015","doi":"10.1002/ajmg.a.36360","title":"Bilateral striatal necrosis in two subjects with Aicardi–Goutières syndrome due to mutations in <i>ADAR1</i> (<i>AGS6</i>)","year":2013,"lang":"en","type":"article","venue":"American Journal of Medical Genetics Part A","topic":"interferon and immune responses","field":"Immunology and Microbiology","cited_by":37,"is_retracted":false,"has_abstract":true,"ca_institutions":"McGill University; Montreal Neurological Institute and Hospital","funders":"European Commission","keywords":"Pathology; Leukoencephalopathy; Medicine; Necrosis; Atrophy; Disease","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":[0.0003359914,0.001472038,0.0006869933,0.001813901,0.001722339,0.0007281066,0.0004687883,0.002138111,0.001534824],"category_scores_gemma":[0.001331053,0.0008358319,0.0005066905,0.0006669543,0.001412171,0.0004533231,0.0009629673,0.0009215288,0.0002765832],"about_ca_system_candidate":false,"about_ca_system_consensus":false,"about_ca_system_score_codex":0.0007320927,"about_ca_system_score_gemma":0.0004526967,"about_ca_topic_candidate":false,"about_ca_topic_consensus":false,"about_ca_topic_score_codex":0.00518584,"about_ca_topic_score_gemma":0.004593602,"domain_scores_codex":[0.9996487,0.00005355082,0.0000408111,0.0001191473,0.00005599445,0.00008185703],"domain_scores_gemma":[0.9994113,0.0001351154,0.0001394871,0.00003980235,0.00005972961,0.0002145877],"domain_codex":null,"domain_gemma":null,"domain_candidate":null,"domain_consensus":null,"study_design_codex":"case_report","study_design_gemma":"case_report","study_design_scores_codex":[0.0005315155,0.0003338929,0.167769,0.00009137353,0.0000864642,0.808413,0.002777915,0.0001419005,0.01513472,0.0002221371,0.0003079871,0.004189951],"study_design_scores_gemma":[0.0001132654,0.0007488769,0.2015575,0.00003059077,0.0000932927,0.7930754,0.000843815,0.0003592519,0.00197533,0.00038395,0.0007687508,0.00004984953],"study_design_candidate":"case_report","study_design_consensus":"case_report","genre_codex":"empirical","genre_gemma":"empirical","genre_scores_codex":[0.9982374,0.0002393977,0.0002517078,0.0001279227,0.00002198779,0.00003205997,0.00003665238,0.0000215016,0.001031352],"genre_scores_gemma":[0.9991139,0.0001255816,0.0002305894,0.0001125193,0.00003777239,0.000009827409,0.00004584268,0.00000846926,0.0003155389],"genre_candidate":"empirical","genre_consensus":"empirical","teacher_disagreement_score":0.00518584,"threshold_uncertainty_score":0.01031131,"prediction_status":"machine_predicted_unvalidated"},"machine_scores":{"provisional":true,"baseline":true,"maturity_gate_passed":false,"score_opus":0.007751739223034166,"score_gpt":0.2629273541032757,"score_spread":0.2551756148802415,"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."}}