{"id":"W2088363799","doi":"10.1074/jbc.m111.221804","title":"Classical and Neonatal Marfan Syndrome Mutations in Fibrillin-1 Cause Differential Protease Susceptibilities and Protein Function","year":2011,"lang":"en","type":"article","venue":"Journal of Biological Chemistry","topic":"Connective tissue disorders research","field":"Biochemistry, Genetics and Molecular Biology","cited_by":54,"is_retracted":false,"has_abstract":true,"ca_institutions":"University of British Columbia; McGill University","funders":"Canadian Institutes of Health Research","keywords":"Fibrillin; Proteases; Mutant; Biology; Chemistry; Extracellular matrix; Biochemistry; Cell biology; Enzyme; Gene","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":"metacan-v3-hybrid-931329e0061c","candidate_categories":[],"consensus_categories":[],"category_scores_codex":[0.0002466241,0.000711152,0.0002753904,0.000554005,0.0001167764,0.0001569663,0.0002600897,0.0005378223,0.001276115],"category_scores_gemma":[0.0009384164,0.0001774104,0.0002177436,0.0001643319,0.0004123417,0.0001896819,0.0003232827,0.000283255,0.0002115164],"about_ca_system_candidate":false,"about_ca_system_consensus":false,"about_ca_system_score_codex":0.0002229444,"about_ca_system_score_gemma":0.00010236,"about_ca_topic_candidate":false,"about_ca_topic_consensus":false,"about_ca_topic_score_codex":0.0002884733,"about_ca_topic_score_gemma":0.000292769,"domain_scores_codex":[0.9996574,0.00005679526,0.00005099102,0.00009054987,0.0001004465,0.00004374974],"domain_scores_gemma":[0.9994439,0.0001236132,0.0002232008,0.00002757873,0.0000345196,0.0001471495],"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":[0.0006630342,0.00006292425,0.02145958,0.00007466583,0.00004135569,0.004658585,0.0001570066,0.0002038843,0.9686338,0.0001960824,0.00006610738,0.003783067],"study_design_scores_gemma":[0.00009947706,0.001360769,0.4891087,0.00005052963,0.0001508769,0.08448331,0.0002873541,0.001971381,0.4198918,0.0004767036,0.002068701,0.00005038902],"study_design_candidate":"bench_or_experimental","study_design_consensus":"bench_or_experimental","genre_codex":"empirical","genre_gemma":"empirical","genre_scores_codex":[0.9989783,0.0003736386,0.0003550473,0.00001328439,0.000003911702,0.000003604233,0.00008738331,0.00001158504,0.0001732326],"genre_scores_gemma":[0.9987966,0.0001832024,0.0006227998,0.00001706988,0.000006653739,0.000009809638,0.0001365824,0.000008663533,0.0002186444],"genre_candidate":"empirical","genre_consensus":"empirical","teacher_disagreement_score":0.001276115,"threshold_uncertainty_score":0.004269004,"prediction_status":"machine_predicted_unvalidated"},"machine_scores":{"provisional":true,"baseline":true,"maturity_gate_passed":false,"score_opus":0.03444883566829082,"score_gpt":0.2679585282206543,"score_spread":0.2335096925523635,"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."}}