{"id":"W2117212431","doi":"10.1016/j.bbadis.2006.06.006","title":"Failure of protein quality control in amyotrophic lateral sclerosis","year":2006,"lang":"en","type":"review","venue":"Biochimica et Biophysica Acta (BBA) - Molecular Basis of Disease","topic":"Amyotrophic Lateral Sclerosis Research","field":"Medicine","cited_by":87,"is_retracted":false,"has_abstract":false,"ca_institutions":"Montreal Neurological Institute and Hospital","funders":"","keywords":"Proteasome; Proteostasis; Protein aggregation; Cell biology; Amyotrophic lateral sclerosis; SOD1; Neurodegeneration; Neuroprotection; Unfolded protein response; Protein folding; Protein degradation; Mutant; Ubiquitin; Endoplasmic-reticulum-associated protein degradation; Chaperone (clinical); Biology; Chemistry; Endoplasmic reticulum; Neuroscience; Biochemistry; Gene; Medicine; 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":"codex-gemma-dda1882f352a","candidate_categories":["metaepi_narrow"],"consensus_categories":[],"category_scores_codex":[0.0006018381,0.001166179,0.004505719,0.0008516614,0.0000588721,0.00006147825,0.0009581432,0.0005915703,0.0000769738],"category_scores_gemma":[0.000382798,0.0009621125,0.002597123,0.001420803,0.0006667278,0.0001632572,0.0003736535,0.001072076,0.00002932838],"about_ca_system_candidate":false,"about_ca_system_consensus":false,"about_ca_system_score_codex":0.000325646,"about_ca_system_score_gemma":0.001395419,"about_ca_topic_candidate":false,"about_ca_topic_consensus":false,"about_ca_topic_score_codex":0.0004461334,"about_ca_topic_score_gemma":0.00001045165,"domain_scores_codex":[0.9918698,0.001564522,0.002427966,0.001412674,0.00175298,0.0009720352],"domain_scores_gemma":[0.9951885,0.0001836403,0.001444836,0.002047006,0.000395437,0.0007405608],"domain_codex":null,"domain_gemma":null,"domain_candidate":null,"domain_consensus":null,"study_design_codex":"bench_or_experimental","study_design_gemma":"systematic_review","study_design_scores_codex":[0.002318122,0.00532341,0.00005809828,0.06302194,0.002023434,0.0002372945,0.00005094659,0.000006328501,0.8998575,0.0004081154,0.0008589515,0.02583583],"study_design_scores_gemma":[0.04925109,0.007183738,0.2051163,0.3954562,0.0303222,0.000039934,0.0000569974,0.0003748965,0.2331181,0.001448853,0.06400547,0.01362618],"study_design_candidate":"bench_or_experimental","study_design_consensus":null,"genre_codex":"empirical","genre_gemma":"empirical","genre_scores_codex":[0.6353847,0.3553287,0.00001761958,0.002575116,0.00004882853,0.004811704,0.001696509,0.00007513781,0.00006169602],"genre_scores_gemma":[0.6924773,0.3051883,0.0003314709,0.0002793198,0.00009148171,0.0005362956,0.0008344693,0.0002070792,0.00005434481],"genre_candidate":"empirical","genre_consensus":"empirical","teacher_disagreement_score":0.6667394,"threshold_uncertainty_score":0.999283,"prediction_status":"machine_predicted_unvalidated"},"machine_scores":{"provisional":true,"baseline":true,"maturity_gate_passed":false,"score_opus":0.04320232546830789,"score_gpt":0.3421937062165098,"score_spread":0.2989913807482019,"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."}}