{"id":"W4376121243","doi":"10.1172/jci.insight.163576","title":"Restoring calcium homeostasis in Purkinje cells arrests neurodegeneration and neuroinflammation in the ARSACS mouse model","year":2023,"lang":"en","type":"article","venue":"JCI Insight","topic":"Genetic Neurodegenerative Diseases","field":"Neuroscience","cited_by":26,"is_retracted":false,"has_abstract":true,"ca_institutions":"McGill University; Montreal Neurological Institute and Hospital","funders":"Fidia Farmaceutici; Ministero della Salute","keywords":"Neuroprotection; Neurodegeneration; Homeostasis; Glutamatergic; Neuroscience; Neuroinflammation; Ataxia; Cell biology; Purkinje cell; Unfolded protein response; Cerebellum; Biology; Downregulation and upregulation; Calcium metabolism; Excitotoxicity; Endoplasmic reticulum; Medicine; Calcium; Programmed cell death; Internal medicine; Apoptosis; Gene; Immunology; Receptor; Genetics; Glutamate receptor; Inflammation","routes":{"ca_aff":true,"ca_fund":false,"ca_venue":false,"about_ca":true,"invisible_to_affiliation_only":false},"retraction":null,"screen":null,"direct_labels":[],"prediction":{"model_version":"metacan-v3-hybrid-931329e0061c","candidate_categories":[],"consensus_categories":[],"category_scores_codex":[0.0003031933,0.001107921,0.0007983305,0.0009980839,0.000318318,0.0004320593,0.0006142714,0.0009256874,0.001666982],"category_scores_gemma":[0.0001491435,0.0003196704,0.000694561,0.0003167916,0.0006721705,0.0005323802,0.0003385334,0.001642547,0.0004431135],"about_ca_system_candidate":false,"about_ca_system_consensus":false,"about_ca_system_score_codex":0.0004370506,"about_ca_system_score_gemma":0.000387754,"about_ca_topic_candidate":false,"about_ca_topic_consensus":false,"about_ca_topic_score_codex":0.0009059522,"about_ca_topic_score_gemma":0.001915829,"domain_scores_codex":[0.9995578,0.00004581697,0.00007280159,0.0001290832,0.0001059363,0.0000885689],"domain_scores_gemma":[0.999643,0.00002085353,0.0001372133,0.0000334092,0.00003375643,0.0001319055],"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":[0.0006379334,0.0001911895,0.0003399674,0.0001251447,0.00002143854,0.0002352658,0.00004774673,0.0001358568,0.99615,0.0001748656,0.0001414117,0.001799059],"study_design_scores_gemma":[0.000231618,0.002650063,0.005929906,0.00005357829,0.00009708642,0.0009466452,0.0000894153,0.002191811,0.9827172,0.0002344222,0.004834241,0.00002402656],"study_design_candidate":"bench_or_experimental","study_design_consensus":"bench_or_experimental","genre_codex":"empirical","genre_gemma":"empirical","genre_scores_codex":[0.9887156,0.002364764,0.00449761,0.0003851986,0.0001198922,0.0001567519,0.001452065,0.0006114081,0.001696805],"genre_scores_gemma":[0.9831877,0.001896481,0.00625288,0.0001473999,0.00004038424,0.0003648955,0.00134762,0.00008352094,0.006679086],"genre_candidate":"empirical","genre_consensus":"empirical","teacher_disagreement_score":0.001666982,"threshold_uncertainty_score":0.00557667,"prediction_status":"machine_predicted_unvalidated"},"machine_scores":{"provisional":true,"baseline":true,"maturity_gate_passed":false,"score_opus":0.07700945842740732,"score_gpt":0.2892909141100591,"score_spread":0.2122814556826518,"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."}}