{"id":"W6991352630","doi":"","title":"Genotype and Phenotype Characterisation of an Enhanced Friedreich Ataxia GAA Repeat Expansion Mouse Model Fxn_null::YG8s(GAA)>800","year":2019,"lang":"en","type":"article","venue":"Brunel University Research Archive (BURA) (Brunel University London)","topic":"Genetic Neurodegenerative Diseases","field":"Neuroscience","cited_by":0,"is_retracted":false,"has_abstract":true,"ca_institutions":"","funders":"College of Medicine, University of Florida; Instituto de Ciências Biomédicas Abel Salazar, Universidade do Porto; Instituto De Saúde Pública, Universidade do Porto; Universidade do Minho; Taipei Medical University; National Yang-Ming University; Eberhard Karls Universität Tübingen; Rigshospitalet; National Institutes of Health; European Commission; Universidade Federal do Rio Grande do Sul; Université de Recherche Paris Sciences et Lettres; Zhejiang University; International Business Machines Corporation; Tel Aviv University; Hokkaido University; Bundesministerium für Bildung und Forschung; Ataxia UK; Taipei Veterans General Hospital; Ministerio de Ciencia, Innovación y Universidades; Tunghai University; McGill University; Universitat Autònoma de Barcelona; Universidade do Porto; Brain Research Center, National Yang-Ming University; Georgetown University; Friedreich's Ataxia Research Alliance","keywords":"Frataxin; Trinucleotide repeat expansion; Genetically modified mouse; Transgene; Ataxia; Exon; Ratón; Loss of heterozygosity; Knockout mouse","routes":{"ca_aff":false,"ca_fund":true,"ca_venue":false,"about_ca":false,"invisible_to_affiliation_only":true},"retraction":null,"screen":null,"direct_labels":[],"prediction":{"model_version":"metacan-v3-hybrid-931329e0061c","candidate_categories":[],"consensus_categories":[],"category_scores_codex":[0.0005916322,0.0006680376,0.0006182584,0.001318709,0.0003709194,0.0004460789,0.0005804656,0.0008357562,0.003487712],"category_scores_gemma":[0.0003367485,0.0004368542,0.0006969695,0.0004026997,0.0004740529,0.0003645165,0.0003594978,0.00158872,0.002705098],"about_ca_system_candidate":false,"about_ca_system_consensus":false,"about_ca_system_score_codex":0.0004564723,"about_ca_system_score_gemma":0.000367806,"about_ca_topic_candidate":false,"about_ca_topic_consensus":false,"about_ca_topic_score_codex":0.001224972,"about_ca_topic_score_gemma":0.002004122,"domain_scores_codex":[0.9994845,0.00003977524,0.00007806309,0.0001299555,0.0001922775,0.00007536881],"domain_scores_gemma":[0.9995511,0.00004316289,0.000114598,0.0000556118,0.00008637203,0.0001491016],"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":[0.0002409935,0.0002187153,0.0009471094,0.00005805777,0.00002194652,0.0004183461,0.00007376267,0.0001689163,0.9928259,0.000423503,0.0004909309,0.004111935],"study_design_scores_gemma":[0.000310746,0.002502228,0.03804315,0.0001092112,0.0002369559,0.01006832,0.0001587482,0.002542521,0.9032409,0.0004045948,0.04229746,0.00008513661],"study_design_candidate":"bench_or_experimental","study_design_consensus":"bench_or_experimental","genre_codex":"empirical","genre_gemma":"empirical","genre_scores_codex":[0.9424642,0.001688525,0.03609036,0.00042275,0.0001035843,0.001043188,0.01145153,0.001012807,0.005722967],"genre_scores_gemma":[0.8440393,0.003795931,0.0753215,0.0004846315,0.00006529311,0.00280421,0.02726877,0.001247696,0.04497274],"genre_candidate":"empirical","genre_consensus":"empirical","teacher_disagreement_score":0.003487712,"threshold_uncertainty_score":0.01166755,"prediction_status":"machine_predicted_unvalidated"},"machine_scores":{"provisional":true,"baseline":true,"maturity_gate_passed":false,"score_opus":0.03310652925876054,"score_gpt":0.2554018614362568,"score_spread":0.2222953321774962,"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."}}