{"doi":"10.1016/j.jneuroim.2016.08.008","title":"Bi-phasic gliosis drives neuropathology in a Sandhoff disease mouse model","abstract":null,"journal":"Journal of Neuroimmunology","year":2016,"id":642137,"datarank":0.26876392038420827,"base_score":1.791759469228055,"endowment":1.791759469228055,"self_citation_contribution":0.26876392038420827,"citation_network_contribution":0.0,"self_endowment_contribution":0.26876392038420827,"citer_contribution":0.0,"corpus_percentile":null,"corpus_rank":null,"citation_count":5,"citer_count":0,"citers_with_citation_signal":0,"citers_with_endowment":0,"datacite_reuse_total":0,"is_dataset":false,"is_dataset_confidence":null,"is_data_producer":false,"deposit_databanks":null,"is_oa":false,"file_count":0,"downloads":0,"has_version_chain":false,"published_date":null,"fair_score":null,"fair_percentile":null,"algorithm_id":"datarank_citation_only_1hop_v6","ranking_scope":"data_only","authors":[{"id":1584314,"name":"Suleiman A. Igdoura","orcid":null,"position":1,"is_corresponding":false},{"id":1670003,"name":"Alexander W.M. Hooper","orcid":null,"position":0,"is_corresponding":false}],"reference_count":0,"raw_metadata":{"has_enrichment":true,"resolved":true,"title":"Bi-phasic gliosis drives neuropathology in a Sandhoff disease mouse model","abstract":"Microgliosis and astrogliosis are known to be exacerbating factors in the progression of the lysosomal storage disorder Sandhoff disease. We have also found evidence for excitotoxicity via glutamate receptors in Sandhoff disease. To view the interaction of these cascades, we measured cerebellar expression of markers for gliosis, apoptosis, and excitatory synapses over the disease course in a Sandhoff disease mouse model. We observe a 2-stage model, with initial activation of microgliosis as early as 60days of age, followed by a later onset of astrogliosis, caspase-mediated apoptosis, and reduction in GluR1 at approximately 100days of age. These results implicate immune cells as first responders in Sandhoff disease.","is_dataset_classified":null,"base_score":0.0,"endowment":0.0,"datacite_reuse_total":0,"file_count":0,"downloads":0,"views":0,"has_version_chain":false,"is_dataset":false,"is_oa":false,"pmid":"27725117","pmcid":null,"openalex_id":null,"authors":[],"funders":[{"funder_name":"Canadian Institute of Health Research","grant_id":"FRN-179714","title":null},{"funder_name":"Canadian Institutes of Health Research","grant_id":"unidentified","title":"unidentified"}],"total_grants":2,"fwci":null,"citation_percentile":null,"influential_citations":0,"citation_trend":[],"oa_status":"closed","license":"Elsevier TDM","oa_locations":[{"url":"https://api.elsevier.com/content/article/PII:S0165572816301862?httpAccept=text/plain","host_type":"publisher"},{"url":"https://api.elsevier.com/content/article/PII:S0165572816301862?httpAccept=text/xml","host_type":"publisher"},{"url":"https://doi.org/10.1016/j.jneuroim.2016.08.008","host_type":""},{"url":"https://pubmed.ncbi.nlm.nih.gov/27725117","host_type":""},{"url":"https://dx.doi.org/10.1016/j.jneuroim.2016.08.008","host_type":""}],"fields_of_study":["0301 basic medicine","0303 health sciences","03 medical and health sciences"],"mesh_terms":["Cerebellum","Animals","Mice, Inbred C57BL","Mice, Knockout","Mice","Sandhoff Disease","Disease Models, Animal","Gliosis","Female","beta-Hexosaminidase beta Chain"],"keywords":["Apoptosis","Glur1","Sandhoff Disease","Astrogliosis","Microgliosis","Cleaved-caspase 9","Mice, Knockout","beta-Hexosaminidase beta Chain","Mice, Inbred C57BL","Disease Models, Animal","Mice","Cerebellum","Animals","Female","Gliosis"],"sdg_mappings":[{"sdg_number":3,"sdg_label":"3. Good health"},{"sdg_number":13,"sdg_label":"13. Climate action"}],"linked_datasets":[],"clinical_trials":[],"software_tools":[],"database_accessions":[],"source":"live","citation_network_status":"fetched"},"created_at":"2026-08-07T21:41:42.283032Z","pmid":null,"pmcid":null,"fwci":null,"citation_percentile":null,"influential_citations":0,"oa_status":null,"license":null,"views":0,"total_file_size_bytes":0,"version_count":0,"fair_f":null,"fair_a":null,"fair_i":null,"fair_r":null,"fair_zscore":null,"fair_rationale":null,"fair_model":null,"fair_agent_version":null,"fair_fulltext_source":null,"fair_has_llm":null,"fair_computed_at":null,"clinical_trials":[],"software_tools":[],"db_accessions":[],"linked_datasets":[],"topics":[]}