{"id":"W2027994069","doi":"10.1007/s00109-006-0046-4","title":"Defective glycosylation of decorin and biglycan, altered collagen structure, and abnormal phenotype of the skin fibroblasts of an Ehlers–Danlos syndrome patient carrying the novel Arg270Cys substitution in galactosyltransferase I (β4GalT-7)","year":2006,"lang":"en","type":"article","venue":"Journal of Molecular Medicine","topic":"Proteoglycans and glycosaminoglycans research","field":"Biochemistry, Genetics and Molecular Biology","cited_by":115,"is_retracted":false,"has_abstract":false,"ca_institutions":"Hospital for Sick Children; University Health Network","funders":"","keywords":"Decorin; Biglycan; Dermatan sulfate; Versican; Proteoglycan; Extracellular matrix; Fibrillogenesis; Chemistry; Molecular biology; Immunoelectron microscopy; Fibroblast; Chondroitin sulfate; Glycosaminoglycan; Cell biology; Biochemistry; Biology; Fibril; In vitro; Immunology; Immunohistochemistry","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":[],"consensus_categories":[],"category_scores_codex":[0.0003879629,0.0001460862,0.0003168994,0.0001499859,0.00004542244,0.000005285485,0.0001707265,0.0001027994,0.000004820878],"category_scores_gemma":[0.0001413307,0.00008887392,0.00006693415,0.0002474265,0.0003399574,0.00001625671,0.0000522154,0.0001502843,1.310611e-8],"about_ca_system_candidate":false,"about_ca_system_consensus":false,"about_ca_system_score_codex":0.00002827613,"about_ca_system_score_gemma":0.0001536529,"about_ca_topic_candidate":false,"about_ca_topic_consensus":false,"about_ca_topic_score_codex":0.000691226,"about_ca_topic_score_gemma":0.0004532484,"domain_scores_codex":[0.9985161,0.0001661828,0.0005800531,0.0001629026,0.0004081862,0.0001666128],"domain_scores_gemma":[0.9989851,0.000037342,0.0004029532,0.0002217052,0.0002864783,0.00006646081],"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":[0.0005336367,0.00007868863,0.006862174,0.00009821197,0.0000651171,0.0000152806,0.0002420049,0.001986472,0.9888623,0.00007732192,0.000003699268,0.001175056],"study_design_scores_gemma":[0.00218282,0.001633021,0.2186055,0.0002306639,0.00006309144,0.0002860964,0.0001329579,0.000317476,0.7762957,0.0001400803,0.00003015115,0.00008250295],"study_design_candidate":"bench_or_experimental","study_design_consensus":"bench_or_experimental","genre_codex":"empirical","genre_gemma":"empirical","genre_scores_codex":[0.9957151,0.001771409,0.001926552,0.00009313566,0.00007113556,0.0003572625,0.00003943284,7.244501e-7,0.00002522529],"genre_scores_gemma":[0.9994935,0.0001009814,0.0002970267,0.00002233209,0.00005609395,0.000002801708,0.00001356303,0.00001187161,0.000001864697],"genre_candidate":"empirical","genre_consensus":"empirical","teacher_disagreement_score":0.2125667,"threshold_uncertainty_score":0.3624173,"prediction_status":"machine_predicted_unvalidated"},"machine_scores":{"provisional":true,"baseline":true,"maturity_gate_passed":false,"score_opus":0.006034924860800029,"score_gpt":0.2309326794525193,"score_spread":0.2248977545917193,"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."}}