{"id":"W2601221906","doi":"10.1016/j.ekir.2017.03.004","title":"The Chemical Chaperone, PBA, Reduces ER Stress and Autophagy and Increases Collagen IV α5 Expression in Cultured Fibroblasts From Men With X-Linked Alport Syndrome and Missense Mutations","year":2017,"lang":"en","type":"article","venue":"Kidney International Reports","topic":"Cell Adhesion Molecules Research","field":"Medicine","cited_by":44,"is_retracted":false,"has_abstract":true,"ca_institutions":"","funders":"Research Councils UK; Kidney Research UK; Kidney Foundation of Canada; Alport Syndrome Foundation; American Society of Nephrology","keywords":"Phenylbutyrate; Immunostaining; Unfolded protein response; Endocrinology; Endoplasmic reticulum; Internal medicine; Molecular biology; ATF6; Medicine; Biology; Biochemistry; Immunohistochemistry","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":[0.0001126813,0.0001746909,0.0001792213,0.0001428641,0.0001652117,0.000187028,0.00009253957,0.0001920958,0.00141495],"category_scores_gemma":[0.000159377,0.0001153129,0.0001508676,0.00007921325,0.0001554999,0.0000737952,0.0001077706,0.0001939951,0.0002190309],"about_ca_system_candidate":false,"about_ca_system_consensus":false,"about_ca_system_score_codex":0.0001121386,"about_ca_system_score_gemma":0.000135182,"about_ca_topic_candidate":false,"about_ca_topic_consensus":false,"about_ca_topic_score_codex":0.0006474454,"about_ca_topic_score_gemma":0.00166865,"domain_scores_codex":[0.999909,0.0000266889,0.000007659985,0.00001838851,0.000022572,0.00001574999],"domain_scores_gemma":[0.9999377,0.00001290638,0.00001599679,0.000005869542,0.000006858775,0.0000206694],"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":[0.000429754,0.00003128731,0.001443763,0.0000477363,0.00002140171,0.00008500183,0.00001895141,0.00002342811,0.9950278,0.00001100088,0.000081867,0.002777962],"study_design_scores_gemma":[0.0001226721,0.002563028,0.05721304,0.00002363463,0.00009350103,0.001647367,0.0001800859,0.0003822808,0.9340569,0.00004101152,0.003668995,0.000007415139],"study_design_candidate":"bench_or_experimental","study_design_consensus":"bench_or_experimental","genre_codex":"empirical","genre_gemma":"empirical","genre_scores_codex":[0.9966894,0.002401941,0.0002647229,0.0001007605,0.00002379495,0.00000746474,0.0001101904,0.00001768285,0.0003839918],"genre_scores_gemma":[0.9972866,0.0007665279,0.0005396091,0.00005510228,0.00001408731,0.00001554636,0.0002114769,0.000005888786,0.001105288],"genre_candidate":"empirical","genre_consensus":"empirical","teacher_disagreement_score":0.00141495,"threshold_uncertainty_score":0.004733503,"prediction_status":"machine_predicted_unvalidated"},"machine_scores":{"provisional":true,"baseline":true,"maturity_gate_passed":false,"score_opus":0.01173287814923209,"score_gpt":0.2902273931933446,"score_spread":0.2784945150441125,"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."}}