{"id":"W4410489539","doi":"10.1007/s12035-025-05057-3","title":"S100B Mitigates Cytoskeletal and Mitochondrial Alterations in a Glial Cell Model of Autosomal Recessive Spastic Ataxia of Charlevoix-Saguenay","year":2025,"lang":"en","type":"article","venue":"Molecular Neurobiology","topic":"Mitochondrial Function and Pathology","field":"Biochemistry, Genetics and Molecular Biology","cited_by":2,"is_retracted":false,"has_abstract":true,"ca_institutions":"","funders":"European Regional Development Fund; Fundação para a Ciência e a Tecnologia; Research Executive Agency; European Commission; Universidade de Lisboa; Ataxia UK","keywords":"Cell biology; Biology; Spinocerebellar ataxia; Intermediate filament; Mitochondrion; Neuroprotection; Protein aggregation; Neurofilament; Cytoskeleton; Ataxia; Cell; Neuroscience; Genetics; Immunology","routes":{"ca_aff":false,"ca_fund":false,"ca_venue":false,"about_ca":true,"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":[0.0001667968,0.0006817026,0.0005711717,0.0004978463,0.0002449929,0.0002589792,0.0003675288,0.000571231,0.0009000066],"category_scores_gemma":[0.00008461603,0.0001552754,0.0002539154,0.0002324338,0.000356135,0.000240561,0.0002321563,0.0008176792,0.000193644],"about_ca_system_candidate":false,"about_ca_system_consensus":false,"about_ca_system_score_codex":0.000297618,"about_ca_system_score_gemma":0.0004092387,"about_ca_topic_candidate":false,"about_ca_topic_consensus":false,"about_ca_topic_score_codex":0.002231778,"about_ca_topic_score_gemma":0.004747648,"domain_scores_codex":[0.9998667,0.00002526711,0.00001463164,0.00002105777,0.00003278053,0.00003945882],"domain_scores_gemma":[0.9998891,0.000005774456,0.00002825895,0.00001119807,0.00001225817,0.00005335712],"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":[0.000897412,0.0003460574,0.0001856576,0.00008588505,0.00002120463,0.0001968917,0.00003534788,0.0001324188,0.9957362,0.000106423,0.0001029794,0.002153519],"study_design_scores_gemma":[0.0002586501,0.007161785,0.009340253,0.0000379672,0.00008923787,0.0004745966,0.000246458,0.002583369,0.975227,0.0001779461,0.004379928,0.00002269243],"study_design_candidate":"bench_or_experimental","study_design_consensus":"bench_or_experimental","genre_codex":"empirical","genre_gemma":"empirical","genre_scores_codex":[0.9972584,0.001045461,0.0006251807,0.0001440641,0.00004679014,0.00002924174,0.0001564956,0.0001048072,0.0005895869],"genre_scores_gemma":[0.9950026,0.001011448,0.0008859931,0.00005266301,0.000008403764,0.00005992709,0.0002805041,0.00001395965,0.002684596],"genre_candidate":"empirical","genre_consensus":"empirical","teacher_disagreement_score":0.002231778,"threshold_uncertainty_score":0.004437566,"prediction_status":"machine_predicted_unvalidated"},"machine_scores":{"provisional":true,"baseline":true,"maturity_gate_passed":false,"score_opus":0.00686920940042742,"score_gpt":0.2303451418894798,"score_spread":0.2234759324890523,"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."}}