{"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":"codex-gemma-dda1882f352a","candidate_categories":[],"consensus_categories":[],"category_scores_codex":[0.0001887212,0.0001532141,0.0002133691,0.00007791823,0.0002365526,0.0001989306,0.00010638,0.00009234522,0.00008767591],"category_scores_gemma":[0.001872725,0.0001004913,0.00002283144,0.00003789273,0.0003439593,0.000159551,0.0002176547,0.0001387064,0.000001314086],"about_ca_system_candidate":false,"about_ca_system_consensus":false,"about_ca_system_score_codex":0.00005179197,"about_ca_system_score_gemma":0.0001875955,"about_ca_topic_candidate":false,"about_ca_topic_consensus":false,"about_ca_topic_score_codex":0.0005386794,"about_ca_topic_score_gemma":0.00005312447,"domain_scores_codex":[0.9984868,0.00003526327,0.0003236423,0.0004334573,0.0005475066,0.0001733507],"domain_scores_gemma":[0.998727,0.0001641921,0.0002385836,0.0003272215,0.0001701283,0.0003728598],"domain_codex":null,"domain_gemma":null,"domain_candidate":null,"domain_consensus":null,"study_design_codex":"bench_or_experimental","study_design_gemma":"observational","study_design_scores_codex":[0.0008377201,0.0002668483,0.1251655,0.00006647083,0.0002456905,0.01192991,0.0009525483,0.00000285277,0.8540373,0.00003200584,0.005105965,0.001357247],"study_design_scores_gemma":[0.002361185,0.0001473222,0.82094,0.001173339,0.00006535651,0.005737389,0.0005291205,0.001133742,0.1648771,0.0003891479,0.002399687,0.0002466563],"study_design_candidate":"bench_or_experimental","study_design_consensus":null,"genre_codex":"empirical","genre_gemma":"empirical","genre_scores_codex":[0.9943594,0.0002666248,0.000004462782,0.002882821,0.00004763384,0.0003840749,0.00004653964,0.0000177149,0.001990738],"genre_scores_gemma":[0.9959529,0.0002182497,0.000885561,0.00007225547,0.0000567208,0.0000501539,0.000203175,0.0000182753,0.002542713],"genre_candidate":"empirical","genre_consensus":"empirical","teacher_disagreement_score":0.6957745,"threshold_uncertainty_score":0.4097916,"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."}}