{"id":"https://openalex.org/W7215967746","doi":"https://doi.org/10.64898/2026.09.27.754840","title":"Hyperexcitable but seizure-free: hippocampal network dysfunction in a Wolfram syndrome model","display_name":"Hyperexcitable but seizure-free: hippocampal network dysfunction in a Wolfram syndrome model","publication_year":2026,"publication_date":"2026-10-02","ids":{"openalex":"https://openalex.org/W7215967746","doi":"https://doi.org/10.64898/2026.09.27.754840","pmid":"https://pubmed.ncbi.nlm.nih.gov/42856230","pmcid":"https://www.ncbi.nlm.nih.gov/pmc/articles/13648544"},"language":"en","primary_location":{"id":"doi:10.64898/2026.09.27.754840","doi":"https://doi.org/10.64898/2026.09.27.754840","is_oa":true,"landing_page_url":"https://doi.org/10.64898/2026.09.27.754840","pdf_url":"https://www.biorxiv.org/content/biorxiv/early/2026/10/02/2026.09.27.754840.full.pdf","source":{"id":"https://openalex.org/S4306402567","display_name":"bioRxiv (Cold Spring Harbor Laboratory)","issn_l":null,"issn":null,"is_oa":false,"is_in_doaj":false,"is_core":false,"listed_in":[],"host_organization":"https://openalex.org/I2750212522","host_organization_name":"Cold Spring Harbor Laboratory","host_organization_lineage":["https://openalex.org/I2750212522"],"host_organization_lineage_names":[],"type":"repository"},"license":"cc-by","license_id":"https://openalex.org/licenses/cc-by","version":"submittedVersion","is_accepted":false,"is_published":false,"raw_source_name":"bioRxiv","raw_type":"posted-content"},"volume":null,"type":"preprint","is_traditional":true,"indexed_in":["crossref","pubmed"],"open_access":{"is_oa":true,"oa_status":"green","oa_url":"https://www.biorxiv.org/content/biorxiv/early/2026/10/02/2026.09.27.754840.full.pdf","any_repository_has_fulltext":true},"authorships":[{"author_position":"first","author":{"id":"https://openalex.org/A5076653492","display_name":"Saumel Ahmadi","orcid":"https://orcid.org/0000-0002-5800-6945","observed_orcids":["https://orcid.org/0000-0002-5800-6945"]},"institutions":[{"id":"https://openalex.org/I204465549","display_name":"Washington University in St. Louis","ror":"https://ror.org/01yc7t268","country_code":"US","type":"education","lineage":["https://openalex.org/I204465549"]}],"countries":["US"],"is_corresponding":false,"raw_author_name":"Saumel Ahmadi","raw_affiliation_strings":["Department of Neurology, Washington University School of Medicine, St. Louis, Missouri, USA"],"raw_orcid":null,"affiliations":[{"raw_affiliation_string":"Department of Neurology, Washington University School of Medicine, St. Louis, Missouri, USA","institution_ids":["https://openalex.org/I204465549"]}]},{"author_position":"middle","author":{"id":"https://openalex.org/A5092601318","display_name":"Juan Gallardo Pinera","orcid":"https://orcid.org/0000-0002-0891-0936","observed_orcids":["https://orcid.org/0000-0002-0891-0936"]},"institutions":[{"id":"https://openalex.org/I204465549","display_name":"Washington University in St. Louis","ror":"https://ror.org/01yc7t268","country_code":"US","type":"education","lineage":["https://openalex.org/I204465549"]}],"countries":["US"],"is_corresponding":false,"raw_author_name":"Juan Gallardo Pinera","raw_affiliation_strings":["Department of Medicine, Division of Endocrinology, Metabolism, and Lipid Research, Washington University School of Medicine, St. Louis, Missouri, USA"],"raw_orcid":null,"affiliations":[{"raw_affiliation_string":"Department of Medicine, Division of Endocrinology, Metabolism, and Lipid Research, Washington University School of Medicine, St. Louis, Missouri, USA","institution_ids":["https://openalex.org/I204465549"]}]},{"author_position":"middle","author":{"id":"https://openalex.org/A5155711459","display_name":"Sophie Dokholyan","orcid":null,"observed_orcids":[]},"institutions":[{"id":"https://openalex.org/I204465549","display_name":"Washington University in St. Louis","ror":"https://ror.org/01yc7t268","country_code":"US","type":"education","lineage":["https://openalex.org/I204465549"]}],"countries":["US"],"is_corresponding":false,"raw_author_name":"Sophie Dokholyan","raw_affiliation_strings":["Department of Neurology, Washington University School of Medicine, St. Louis, Missouri, USA"],"raw_orcid":null,"affiliations":[{"raw_affiliation_string":"Department of Neurology, Washington University School of Medicine, St. Louis, Missouri, USA","institution_ids":["https://openalex.org/I204465549"]}]},{"author_position":"middle","author":{"id":"https://openalex.org/A5024352258","display_name":"Andrew D. Sauerbeck","orcid":"https://orcid.org/0000-0002-4586-0579","observed_orcids":["https://orcid.org/0000-0002-4586-0579"]},"institutions":[{"id":"https://openalex.org/I204465549","display_name":"Washington University in St. Louis","ror":"https://ror.org/01yc7t268","country_code":"US","type":"education","lineage":["https://openalex.org/I204465549"]}],"countries":["US"],"is_corresponding":false,"raw_author_name":"Andrew Sauerbeck","raw_affiliation_strings":["Department of Neurology, Washington University School of Medicine, St. Louis, Missouri, USA"],"raw_orcid":null,"affiliations":[{"raw_affiliation_string":"Department of Neurology, Washington University School of Medicine, St. Louis, Missouri, USA","institution_ids":["https://openalex.org/I204465549"]}]},{"author_position":"middle","author":{"id":"https://openalex.org/A5156294052","display_name":"Hong Nhung Phan","orcid":null,"observed_orcids":[]},"institutions":[{"id":"https://openalex.org/I204465549","display_name":"Washington University in St. Louis","ror":"https://ror.org/01yc7t268","country_code":"US","type":"education","lineage":["https://openalex.org/I204465549"]}],"countries":["US"],"is_corresponding":false,"raw_author_name":"Hong Nhung Phan","raw_affiliation_strings":["Department of Medicine, Division of Endocrinology, Metabolism, and Lipid Research, Washington University School of Medicine, St. Louis, Missouri, USA"],"raw_orcid":null,"affiliations":[{"raw_affiliation_string":"Department of Medicine, Division of Endocrinology, Metabolism, and Lipid Research, Washington University School of Medicine, St. Louis, Missouri, USA","institution_ids":["https://openalex.org/I204465549"]}]},{"author_position":"middle","author":{"id":"https://openalex.org/A5094021133","display_name":"Brianna Carman","orcid":null,"observed_orcids":[]},"institutions":[{"id":"https://openalex.org/I204465549","display_name":"Washington University in St. Louis","ror":"https://ror.org/01yc7t268","country_code":"US","type":"education","lineage":["https://openalex.org/I204465549"]}],"countries":["US"],"is_corresponding":false,"raw_author_name":"Brianna Carman","raw_affiliation_strings":["Department of Medicine, Division of Endocrinology, Metabolism, and Lipid Research, Washington University School of Medicine, St. Louis, Missouri, USA"],"raw_orcid":null,"affiliations":[{"raw_affiliation_string":"Department of Medicine, Division of Endocrinology, Metabolism, and Lipid Research, Washington University School of Medicine, St. Louis, Missouri, USA","institution_ids":["https://openalex.org/I204465549"]}]},{"author_position":"middle","author":{"id":"https://openalex.org/A5033692273","display_name":"Avishek Debnath","orcid":"https://orcid.org/0009-0008-6374-2577","observed_orcids":["https://orcid.org/0009-0008-6374-2577"]},"institutions":[{"id":"https://openalex.org/I204465549","display_name":"Washington University in St. Louis","ror":"https://ror.org/01yc7t268","country_code":"US","type":"education","lineage":["https://openalex.org/I204465549"]}],"countries":["US"],"is_corresponding":false,"raw_author_name":"Avishek Debnath","raw_affiliation_strings":["Department of Engineering - Mechanical Engineering & Materials Science, Washington University School of Medicine, St. Louis, Missouri, USA"],"raw_orcid":null,"affiliations":[{"raw_affiliation_string":"Department of Engineering - Mechanical Engineering & Materials Science, Washington University School of Medicine, St. Louis, Missouri, USA","institution_ids":["https://openalex.org/I204465549"]}]},{"author_position":"middle","author":{"id":"https://openalex.org/A5154378668","display_name":"Samuel Brunwasser","orcid":null,"observed_orcids":[]},"institutions":[{"id":"https://openalex.org/I180670191","display_name":"University of California, San Francisco","ror":"https://ror.org/043mz5j54","country_code":"US","type":"education","lineage":["https://openalex.org/I180670191"]}],"countries":["US"],"is_corresponding":false,"raw_author_name":"Samuel Brunwasser","raw_affiliation_strings":["Department of Neurology, University of California San Francisco, San Francisco, California, USA"],"raw_orcid":null,"affiliations":[{"raw_affiliation_string":"Department of Neurology, University of California San Francisco, San Francisco, California, USA","institution_ids":["https://openalex.org/I180670191"]}]},{"author_position":"middle","author":{"id":"https://openalex.org/A5116140795","display_name":"Gabriel Skinner","orcid":null,"observed_orcids":[]},"institutions":[{"id":"https://openalex.org/I204465549","display_name":"Washington University in St. Louis","ror":"https://ror.org/01yc7t268","country_code":"US","type":"education","lineage":["https://openalex.org/I204465549"]}],"countries":["US"],"is_corresponding":false,"raw_author_name":"Gabriel Skinner","raw_affiliation_strings":["Department of Medicine, Division of Endocrinology, Metabolism, and Lipid Research, Washington University School of Medicine, St. Louis, Missouri, USA"],"raw_orcid":null,"affiliations":[{"raw_affiliation_string":"Department of Medicine, Division of Endocrinology, Metabolism, and Lipid Research, Washington University School of Medicine, St. Louis, Missouri, USA","institution_ids":["https://openalex.org/I204465549"]}]},{"author_position":"middle","author":{"id":"https://openalex.org/A5038980267","display_name":"Nicholas R. Rensing","orcid":null,"observed_orcids":[]},"institutions":[{"id":"https://openalex.org/I204465549","display_name":"Washington University in St. Louis","ror":"https://ror.org/01yc7t268","country_code":"US","type":"education","lineage":["https://openalex.org/I204465549"]}],"countries":["US"],"is_corresponding":false,"raw_author_name":"Nicholas Rensing","raw_affiliation_strings":["Department of Neurology, Washington University School of Medicine, St. Louis, Missouri, USA"],"raw_orcid":null,"affiliations":[{"raw_affiliation_string":"Department of Neurology, Washington University School of Medicine, St. Louis, Missouri, USA","institution_ids":["https://openalex.org/I204465549"]}]},{"author_position":"middle","author":{"id":"https://openalex.org/A5133628146","display_name":"Michael Wong","orcid":"https://orcid.org/0000-0002-3796-743X","observed_orcids":["https://orcid.org/0000-0002-3796-743X"]},"institutions":[{"id":"https://openalex.org/I204465549","display_name":"Washington University in St. Louis","ror":"https://ror.org/01yc7t268","country_code":"US","type":"education","lineage":["https://openalex.org/I204465549"]}],"countries":["US"],"is_corresponding":false,"raw_author_name":"Michael Wong","raw_affiliation_strings":["Department of Neurology, Washington University School of Medicine, St. Louis, Missouri, USA"],"raw_orcid":null,"affiliations":[{"raw_affiliation_string":"Department of Neurology, Washington University School of Medicine, St. Louis, Missouri, USA","institution_ids":["https://openalex.org/I204465549"]}]},{"author_position":"middle","author":{"id":"https://openalex.org/A5120784764","display_name":"Cory Cearlock","orcid":"https://orcid.org/0009-0000-9588-1370","observed_orcids":["https://orcid.org/0009-0000-9588-1370"]},"institutions":[{"id":"https://openalex.org/I204465549","display_name":"Washington University in St. Louis","ror":"https://ror.org/01yc7t268","country_code":"US","type":"education","lineage":["https://openalex.org/I204465549"]}],"countries":["US"],"is_corresponding":false,"raw_author_name":"Cory Cearlock","raw_affiliation_strings":["Department of Psychiatry, Washington University School of Medicine, St. Louis, Missouri, USA","Intellectual and Developmental Disabilities Research Center, Washington University School of Medicine, St. Louis, Missouri, USA"],"raw_orcid":null,"affiliations":[{"raw_affiliation_string":"Department of Psychiatry, Washington University School of Medicine, St. Louis, Missouri, USA","institution_ids":["https://openalex.org/I204465549"]},{"raw_affiliation_string":"Intellectual and Developmental Disabilities Research Center, Washington University School of Medicine, St. Louis, Missouri, USA","institution_ids":["https://openalex.org/I204465549"]}]},{"author_position":"middle","author":{"id":"https://openalex.org/A5024056019","display_name":"Susan E. Maloney","orcid":"https://orcid.org/0000-0001-5585-4722","observed_orcids":["https://orcid.org/0000-0001-5585-4722"]},"institutions":[{"id":"https://openalex.org/I204465549","display_name":"Washington University in St. Louis","ror":"https://ror.org/01yc7t268","country_code":"US","type":"education","lineage":["https://openalex.org/I204465549"]}],"countries":["US"],"is_corresponding":false,"raw_author_name":"Susan E. Maloney","raw_affiliation_strings":["Department of Psychiatry, Washington University School of Medicine, St. Louis, Missouri, USA","Intellectual and Developmental Disabilities Research Center, Washington University School of Medicine, St. Louis, Missouri, USA"],"raw_orcid":"https://orcid.org/0000-0001-5585-4722","affiliations":[{"raw_affiliation_string":"Department of Psychiatry, Washington University School of Medicine, St. Louis, Missouri, USA","institution_ids":["https://openalex.org/I204465549"]},{"raw_affiliation_string":"Intellectual and Developmental Disabilities Research Center, Washington University School of Medicine, St. Louis, Missouri, USA","institution_ids":["https://openalex.org/I204465549"]}]},{"author_position":"middle","author":{"id":"https://openalex.org/A5040525760","display_name":"Terrance T. Kummer","orcid":"https://orcid.org/0000-0001-8938-8280","observed_orcids":["https://orcid.org/0000-0001-8938-8280"]},"institutions":[{"id":"https://openalex.org/I204465549","display_name":"Washington University in St. Louis","ror":"https://ror.org/01yc7t268","country_code":"US","type":"education","lineage":["https://openalex.org/I204465549"]}],"countries":["US"],"is_corresponding":false,"raw_author_name":"Terrance Kummer","raw_affiliation_strings":["Department of Neurology, Washington University School of Medicine, St. Louis, Missouri, USA"],"raw_orcid":null,"affiliations":[{"raw_affiliation_string":"Department of Neurology, Washington University School of Medicine, St. Louis, Missouri, USA","institution_ids":["https://openalex.org/I204465549"]}]},{"author_position":"middle","author":{"id":"https://openalex.org/A5108359938","display_name":"Srikanth Singamaneni","orcid":null,"observed_orcids":[]},"institutions":[{"id":"https://openalex.org/I204465549","display_name":"Washington University in St. Louis","ror":"https://ror.org/01yc7t268","country_code":"US","type":"education","lineage":["https://openalex.org/I204465549"]}],"countries":["US"],"is_corresponding":false,"raw_author_name":"Srikanth Singamaneni","raw_affiliation_strings":["Department of Engineering - Mechanical Engineering & Materials Science, Washington University School of Medicine, St. Louis, Missouri, USA"],"raw_orcid":null,"affiliations":[{"raw_affiliation_string":"Department of Engineering - Mechanical Engineering & Materials Science, Washington University School of Medicine, St. Louis, Missouri, USA","institution_ids":["https://openalex.org/I204465549"]}]},{"author_position":"middle","author":{"id":"https://openalex.org/A5086615705","display_name":"John R. Cirrito","orcid":"https://orcid.org/0000-0002-7196-8786","observed_orcids":["https://orcid.org/0000-0002-7196-8786"]},"institutions":[{"id":"https://openalex.org/I204465549","display_name":"Washington University in St. Louis","ror":"https://ror.org/01yc7t268","country_code":"US","type":"education","lineage":["https://openalex.org/I204465549"]}],"countries":["US"],"is_corresponding":false,"raw_author_name":"John Cirrito","raw_affiliation_strings":["Department of Neurology, Washington University School of Medicine, St. Louis, Missouri, USA"],"raw_orcid":null,"affiliations":[{"raw_affiliation_string":"Department of Neurology, Washington University School of Medicine, St. Louis, Missouri, USA","institution_ids":["https://openalex.org/I204465549"]}]},{"author_position":"middle","author":{"id":"https://openalex.org/A5012491658","display_name":"Thomas J. Foutz","orcid":"https://orcid.org/0000-0002-0114-0019","observed_orcids":["https://orcid.org/0000-0002-0114-0019"]},"institutions":[{"id":"https://openalex.org/I204465549","display_name":"Washington University in St. Louis","ror":"https://ror.org/01yc7t268","country_code":"US","type":"education","lineage":["https://openalex.org/I204465549"]}],"countries":["US"],"is_corresponding":false,"raw_author_name":"Thomas Foutz","raw_affiliation_strings":["Department of Neurology, Washington University School of Medicine, St. Louis, Missouri, USA"],"raw_orcid":"https://orcid.org/0000-0002-0114-0019","affiliations":[{"raw_affiliation_string":"Department of Neurology, Washington University School of Medicine, St. Louis, Missouri, USA","institution_ids":["https://openalex.org/I204465549"]}]},{"author_position":"last","author":{"id":"https://openalex.org/A5006017994","display_name":"Fumihiko Urano","orcid":"https://orcid.org/0000-0002-9944-6293","observed_orcids":["https://orcid.org/0000-0002-9944-6293"]},"institutions":[{"id":"https://openalex.org/I204465549","display_name":"Washington University in St. Louis","ror":"https://ror.org/01yc7t268","country_code":"US","type":"education","lineage":["https://openalex.org/I204465549"]}],"countries":["US"],"is_corresponding":true,"raw_author_name":"Fumihiko Urano","raw_affiliation_strings":["Department of Medicine, Division of Endocrinology, Metabolism, and Lipid Research, Washington University School of Medicine, St. Louis, Missouri, USA","Department of Pathology & Immunology, Washington University School of Medicine, St. Louis, Missouri, USA"],"raw_orcid":"https://orcid.org/0000-0002-9944-6293","affiliations":[{"raw_affiliation_string":"Department of Medicine, Division of Endocrinology, Metabolism, and Lipid Research, Washington University School of Medicine, St. Louis, Missouri, USA","institution_ids":["https://openalex.org/I204465549"]},{"raw_affiliation_string":"Department of Pathology & Immunology, Washington University School of Medicine, St. Louis, Missouri, USA","institution_ids":["https://openalex.org/I204465549"]}]}],"institutions":[],"countries_distinct_count":1,"institutions_distinct_count":2,"corresponding_author_ids":["https://openalex.org/A5006017994"],"corresponding_institution_ids":["https://openalex.org/I204465549"],"apc_list":null,"apc_paid":null,"fwci":null,"has_fulltext":true,"cited_by_count":0,"citation_normalized_percentile":null,"cited_by_percentile_year":null,"biblio":{"volume":null,"issue":null,"first_page":null,"last_page":null},"is_retracted":false,"is_paratext":false,"is_xpac":false,"primary_topic":{"id":"https://openalex.org/T11150","display_name":"Endoplasmic Reticulum Stress and Disease","score":0.6265000104904175,"subfield":{"id":"https://openalex.org/subfields/1307","display_name":"Cell Biology"},"field":{"id":"https://openalex.org/fields/13","display_name":"Biochemistry, Genetics and Molecular Biology"},"domain":{"id":"https://openalex.org/domains/1","display_name":"Life Sciences"}},"topics":[{"id":"https://openalex.org/T11150","display_name":"Endoplasmic Reticulum Stress and Disease","score":0.6265000104904175,"subfield":{"id":"https://openalex.org/subfields/1307","display_name":"Cell Biology"},"field":{"id":"https://openalex.org/fields/13","display_name":"Biochemistry, Genetics and Molecular Biology"},"domain":{"id":"https://openalex.org/domains/1","display_name":"Life Sciences"}},{"id":"https://openalex.org/T10094","display_name":"Epilepsy research and treatment","score":0.2655999958515167,"subfield":{"id":"https://openalex.org/subfields/2728","display_name":"Clinical Neurology"},"field":{"id":"https://openalex.org/fields/27","display_name":"Medicine"},"domain":{"id":"https://openalex.org/domains/4","display_name":"Health Sciences"}},{"id":"https://openalex.org/T11772","display_name":"Genetics and Neurodevelopmental Disorders","score":0.032099999487400055,"subfield":{"id":"https://openalex.org/subfields/1311","display_name":"Genetics"},"field":{"id":"https://openalex.org/fields/13","display_name":"Biochemistry, Genetics and Molecular Biology"},"domain":{"id":"https://openalex.org/domains/1","display_name":"Life Sciences"}}],"keywords":[{"id":"https://openalex.org/keywords/wolfram-syndrome","display_name":"Wolfram syndrome","score":1.0},{"id":"https://openalex.org/keywords/wfs1","display_name":"WFS1","score":0.847000002861023},{"id":"https://openalex.org/keywords/hippocampus","display_name":"hippocampus","score":0.8920000195503235},{"id":"https://openalex.org/keywords/neuronal-hyperexcitability","display_name":"neuronal hyperexcitability","score":0.9610000252723694},{"id":"https://openalex.org/keywords/endoplasmic-reticulum-stress","display_name":"endoplasmic reticulum stress","score":0.9070000052452087},{"id":"https://openalex.org/keywords/network-bursting","display_name":"network bursting","score":0.7889999747276306},{"id":"https://openalex.org/keywords/network-criticality","display_name":"network criticality","score":0.8560000061988831},{"id":"https://openalex.org/keywords/sex-differences","display_name":"sex differences","score":0.6740000247955322},{"id":"https://openalex.org/keywords/sleep-disruption","display_name":"sleep disruption","score":0.6589999794960022},{"id":"https://openalex.org/keywords/fear-memory","display_name":"fear memory","score":0.7599999904632568},{"id":"https://openalex.org/keywords/motor-activity","display_name":"motor activity","score":0.6740000247955322},{"id":"https://openalex.org/keywords/epileptiform-activity","display_name":"epileptiform activity","score":0.7229999899864197},{"id":"https://openalex.org/keywords/mouse-model","display_name":"mouse model","score":0.3959999978542328}],"concepts":[{"id":"https://openalex.org/C169760540","wikidata":"https://www.wikidata.org/wiki/Q207011","display_name":"Neuroscience","level":1,"score":0.8518000245094299},{"id":"https://openalex.org/C148762608","wikidata":"https://www.wikidata.org/wiki/Q3748185","display_name":"Hippocampal formation","level":2,"score":0.8248000144958496},{"id":"https://openalex.org/C112592302","wikidata":"https://www.wikidata.org/wiki/Q1207387","display_name":"Excitatory postsynaptic potential","level":3,"score":0.6481999754905701},{"id":"https://openalex.org/C2781161787","wikidata":"https://www.wikidata.org/wiki/Q48360","display_name":"Hippocampus","level":2,"score":0.5200999975204468},{"id":"https://openalex.org/C195221683","wikidata":"https://www.wikidata.org/wiki/Q5000649","display_name":"Bursting","level":2,"score":0.49950000643730164},{"id":"https://openalex.org/C2908647359","wikidata":"https://www.wikidata.org/wiki/Q2625603","display_name":"Population","level":2,"score":0.4763000011444092},{"id":"https://openalex.org/C86803240","wikidata":"https://www.wikidata.org/wiki/Q420","display_name":"Biology","level":0,"score":0.4300000071525574},{"id":"https://openalex.org/C127716648","wikidata":"https://www.wikidata.org/wiki/Q104053","display_name":"Phenotype","level":3,"score":0.3346000015735626},{"id":"https://openalex.org/C25274449","wikidata":"https://www.wikidata.org/wiki/Q1805481","display_name":"Long-term potentiation","level":3,"score":0.32109999656677246},{"id":"https://openalex.org/C71924100","wikidata":"https://www.wikidata.org/wiki/Q11190","display_name":"Medicine","level":0,"score":0.31150001287460327},{"id":"https://openalex.org/C88576662","wikidata":"https://www.wikidata.org/wiki/Q18646","display_name":"Episodic memory","level":3,"score":0.30300000309944153},{"id":"https://openalex.org/C2778186239","wikidata":"https://www.wikidata.org/wiki/Q41571","display_name":"Epilepsy","level":2,"score":0.2962000072002411},{"id":"https://openalex.org/C2776925932","wikidata":"https://www.wikidata.org/wiki/Q1755122","display_name":"Neurodegeneration","level":3,"score":0.29319998621940613},{"id":"https://openalex.org/C185263204","wikidata":"https://www.wikidata.org/wiki/Q1154774","display_name":"Electrophysiology","level":2,"score":0.2833999991416931},{"id":"https://openalex.org/C15744967","wikidata":"https://www.wikidata.org/wiki/Q9418","display_name":"Psychology","level":0,"score":0.2630999982357025},{"id":"https://openalex.org/C2779746338","wikidata":"https://www.wikidata.org/wiki/Q6337803","display_name":"Neuroscientist","level":5,"score":0.26260000467300415},{"id":"https://openalex.org/C17077164","wikidata":"https://www.wikidata.org/wiki/Q1185869","display_name":"Inhibitory postsynaptic potential","level":2,"score":0.2623000144958496}],"mesh":[],"locations_count":3,"locations":[{"id":"doi:10.64898/2026.09.27.754840","doi":"https://doi.org/10.64898/2026.09.27.754840","is_oa":true,"landing_page_url":"https://doi.org/10.64898/2026.09.27.754840","pdf_url":"https://www.biorxiv.org/content/biorxiv/early/2026/10/02/2026.09.27.754840.full.pdf","source":{"id":"https://openalex.org/S4306402567","display_name":"bioRxiv (Cold Spring Harbor Laboratory)","issn_l":null,"issn":null,"is_oa":false,"is_in_doaj":false,"is_core":false,"listed_in":[],"host_organization":"https://openalex.org/I2750212522","host_organization_name":"Cold Spring Harbor Laboratory","host_organization_lineage":["https://openalex.org/I2750212522"],"host_organization_lineage_names":[],"type":"repository"},"license":"cc-by","license_id":"https://openalex.org/licenses/cc-by","version":"submittedVersion","is_accepted":false,"is_published":false,"raw_source_name":"bioRxiv","raw_type":"posted-content"},{"id":"pmh:oai:pubmedcentral.nih.gov:13648544","doi":null,"is_oa":true,"landing_page_url":"https://pmc.ncbi.nlm.nih.gov/articles/PMC13648544/","pdf_url":"https://pmc.ncbi.nlm.nih.gov/articles/PMC13648544/pdf/nihpp-2026.09.27.754840v1.pdf","source":{"id":"https://openalex.org/S2764455111","display_name":"PubMed Central","issn_l":null,"issn":null,"is_oa":true,"is_in_doaj":false,"is_core":false,"listed_in":[],"host_organization":"https://openalex.org/I1299303238","host_organization_name":"National Institutes of Health","host_organization_lineage":["https://openalex.org/I1299303238"],"host_organization_lineage_names":[],"type":"repository"},"license":"cc-by","license_id":"https://openalex.org/licenses/cc-by","version":"publishedVersion","is_accepted":true,"is_published":true,"raw_source_name":"bioRxiv","raw_type":"Text"},{"id":"pmid:42856230","doi":null,"is_oa":false,"landing_page_url":"https://pubmed.ncbi.nlm.nih.gov/42856230","pdf_url":null,"source":{"id":"https://openalex.org/S4306525036","display_name":"PubMed","issn_l":null,"issn":null,"is_oa":false,"is_in_doaj":false,"is_core":false,"listed_in":[],"host_organization":"https://openalex.org/I1299303238","host_organization_name":"National Institutes of Health","host_organization_lineage":["https://openalex.org/I1299303238"],"host_organization_lineage_names":[],"type":"repository"},"license":null,"license_id":null,"version":"publishedVersion","is_accepted":true,"is_published":true,"raw_source_name":"bioRxiv : the preprint server for biology","raw_type":"Journal Article"}],"best_oa_location":{"id":"doi:10.64898/2026.09.27.754840","doi":"https://doi.org/10.64898/2026.09.27.754840","is_oa":true,"landing_page_url":"https://doi.org/10.64898/2026.09.27.754840","pdf_url":"https://www.biorxiv.org/content/biorxiv/early/2026/10/02/2026.09.27.754840.full.pdf","source":{"id":"https://openalex.org/S4306402567","display_name":"bioRxiv (Cold Spring Harbor Laboratory)","issn_l":null,"issn":null,"is_oa":false,"is_in_doaj":false,"is_core":false,"listed_in":[],"host_organization":"https://openalex.org/I2750212522","host_organization_name":"Cold Spring Harbor Laboratory","host_organization_lineage":["https://openalex.org/I2750212522"],"host_organization_lineage_names":[],"type":"repository"},"license":"cc-by","license_id":"https://openalex.org/licenses/cc-by","version":"submittedVersion","is_accepted":false,"is_published":false,"raw_source_name":"bioRxiv","raw_type":"posted-content"},"sustainable_development_goals":[{"display_name":"Good health and well-being","id":"https://metadata.un.org/sdg/3","score":0.8371999859809875}],"study_designs":[],"awards":[{"id":"https://openalex.org/G1964889935","display_name":"Diabetes Research & Training Center","funder_award_id":"5p60dk020579-35","funder_id":"https://openalex.org/F4320332161","funder_display_name":"National Institutes of Health"},{"id":"https://openalex.org/G2139114335","display_name":null,"funder_award_id":"HD103525","funder_id":"https://openalex.org/F4320338053","funder_display_name":"Intellectual and Developmental Disabilities Research Center, Washington University School of Medicine in St. Louis"},{"id":"https://openalex.org/G396128773","display_name":"Washington University Intellectual and Developmental Disabilities Research Center","funder_award_id":"P50HD103525","funder_id":"https://openalex.org/F4320337611","funder_display_name":"Eunice Kennedy Shriver National Institute of Child Health and Human Development"},{"id":"https://openalex.org/G412530589","display_name":null,"funder_award_id":"P30 DK020579","funder_id":"https://openalex.org/F4320306076","funder_display_name":"National Science Foundation"},{"id":"https://openalex.org/G5974922019","display_name":null,"funder_award_id":"2331330","funder_id":"https://openalex.org/F4320337390","funder_display_name":"Division of Chemical, Bioengineering, Environmental, and Transport Systems"},{"id":"https://openalex.org/G8276238797","display_name":null,"funder_award_id":"P50 HD103525","funder_id":"https://openalex.org/F4320338053","funder_display_name":"Intellectual and Developmental Disabilities Research Center, Washington University School of Medicine in St. Louis"},{"id":"https://openalex.org/G8287076523","display_name":"Understanding Fundamental Mechanisms that Underlie Nano-Neuro Interactions","funder_award_id":"2331330","funder_id":"https://openalex.org/F4320306076","funder_display_name":"National Science Foundation"},{"id":"https://openalex.org/G9000153645","display_name":null,"funder_award_id":"DK020579","funder_id":"https://openalex.org/F4320332161","funder_display_name":"National Institutes of Health"},{"id":"https://openalex.org/G9000894439","display_name":"Washington University Institute of Clinical and Translational Sciences (KL2)","funder_award_id":"KL2TR002346","funder_id":"https://openalex.org/F4320332161","funder_display_name":"National Institutes of Health"},{"id":"https://openalex.org/G9000898797","display_name":"Diabetes Research Center","funder_award_id":"P30DK020579","funder_id":"https://openalex.org/F4320337357","funder_display_name":"National Institute of Diabetes and Digestive and Kidney Diseases"},{"id":"https://openalex.org/G9000909700","display_name":"Molecular Genetics of Hereditary Endoplasmic Reticulum Diabetes","funder_award_id":"R01DK132090","funder_id":"https://openalex.org/F4320337357","funder_display_name":"National Institute of Diabetes and Digestive and Kidney Diseases"}],"funders":[{"id":"https://openalex.org/F4320306076","display_name":"National Science Foundation","ror":"https://ror.org/021nxhr62"},{"id":"https://openalex.org/F4320332161","display_name":"National Institutes of Health","ror":"https://ror.org/01cwqze88"},{"id":"https://openalex.org/F4320337357","display_name":"National Institute of Diabetes and Digestive and Kidney Diseases","ror":"https://ror.org/00adh9b73"},{"id":"https://openalex.org/F4320337390","display_name":"Division of Chemical, Bioengineering, Environmental, and Transport Systems","ror":"https://ror.org/0471zv972"},{"id":"https://openalex.org/F4320337611","display_name":"Eunice Kennedy Shriver National Institute of Child Health and Human Development","ror":"https://ror.org/04byxyr05"},{"id":"https://openalex.org/F4320338053","display_name":"Intellectual and Developmental Disabilities Research Center, Washington University School of Medicine in St. Louis","ror":null}],"has_content":{"pdf":true,"grobid_xml":true},"content_urls":{"pdf":"https://content.openalex.org/works/W7215967746.pdf","grobid_xml":"https://content.openalex.org/works/W7215967746.grobid-xml"},"referenced_works_count":89,"referenced_works":["https://openalex.org/W1550524407","https://openalex.org/W1736719008","https://openalex.org/W1966173885","https://openalex.org/W1985648546","https://openalex.org/W1990752558","https://openalex.org/W1997720138","https://openalex.org/W2000344096","https://openalex.org/W2001954021","https://openalex.org/W2010344395","https://openalex.org/W2015513895","https://openalex.org/W2029601828","https://openalex.org/W2044215073","https://openalex.org/W2046203847","https://openalex.org/W2048172511","https://openalex.org/W2051526952","https://openalex.org/W2056313099","https://openalex.org/W2057250808","https://openalex.org/W2063750361","https://openalex.org/W2068699946","https://openalex.org/W2085602278","https://openalex.org/W2089640650","https://openalex.org/W2095265457","https://openalex.org/W2104463303","https://openalex.org/W2107349501","https://openalex.org/W2111205934","https://openalex.org/W2113981430","https://openalex.org/W2116657339","https://openalex.org/W2120122995","https://openalex.org/W2122803078","https://openalex.org/W2123194540","https://openalex.org/W2124745094","https://openalex.org/W2125737923","https://openalex.org/W2138872551","https://openalex.org/W2140319789","https://openalex.org/W2141041915","https://openalex.org/W2171668474","https://openalex.org/W2234697191","https://openalex.org/W2312558124","https://openalex.org/W2481823524","https://openalex.org/W2490214293","https://openalex.org/W2571120430","https://openalex.org/W2739169649","https://openalex.org/W2746678792","https://openalex.org/W2783481291","https://openalex.org/W2788135418","https://openalex.org/W2806654158","https://openalex.org/W2902494615","https://openalex.org/W2935859875","https://openalex.org/W2951614919","https://openalex.org/W2952442557","https://openalex.org/W2955438150","https://openalex.org/W2963909599","https://openalex.org/W3006342576","https://openalex.org/W3010667279","https://openalex.org/W3022125061","https://openalex.org/W3040593374","https://openalex.org/W3048056023","https://openalex.org/W3093181376","https://openalex.org/W3097577171","https://openalex.org/W3110151578","https://openalex.org/W3120232967","https://openalex.org/W3138843227","https://openalex.org/W3176663500","https://openalex.org/W3201262563","https://openalex.org/W3203099629","https://openalex.org/W4205367823","https://openalex.org/W4214601476","https://openalex.org/W4220892317","https://openalex.org/W4226257521","https://openalex.org/W4294677402","https://openalex.org/W4297051734","https://openalex.org/W4319437999","https://openalex.org/W4353032056","https://openalex.org/W4381664147","https://openalex.org/W4382344884","https://openalex.org/W4382931541","https://openalex.org/W4386262048","https://openalex.org/W4390938746","https://openalex.org/W4394883221","https://openalex.org/W4400863348","https://openalex.org/W4401462193","https://openalex.org/W4403718978","https://openalex.org/W4405711794","https://openalex.org/W4411160887","https://openalex.org/W4417359535","https://openalex.org/W7125689463","https://openalex.org/W7161410486","https://openalex.org/W7167088840","https://openalex.org/W7168281040"],"related_works":["https://openalex.org/W4389261571","https://openalex.org/W2063750361","https://openalex.org/W2788135418","https://openalex.org/W3204997440","https://openalex.org/W2282989910","https://openalex.org/W2014146179","https://openalex.org/W3157320940","https://openalex.org/W4220906758","https://openalex.org/W1892490551","https://openalex.org/W2745694328"],"abstract_inverted_index":{"Wolfram":[0],"syndrome":[1],"(WS)":[2],"is":[3,19,41,105],"a":[4,20,66,136,146,153,200],"rare,":[5],"life-limiting":[6],"progressive":[7],"neurodegenerative":[8],"disorder":[9],"caused":[10],"by":[11],"pathogenic":[12],"variants":[13],"in":[14,33,43,51,63,91,97,115,128,139,177],"the":[15,28,44,46,178,204],"WFS1":[16,39],"gene.":[17],"It":[18],"prototype":[21],"endoplasmic":[22],"reticulum":[23],"(ER)":[24],"stress":[25],"disorder,":[26],"and":[27,80,94,165],"mechanisms":[29],"underlying":[30],"neurological":[31,205],"phenotypes":[32],"WS":[34,52,179],"remain":[35],"incompletely":[36],"understood.":[37],"Although":[38],"protein":[40],"abundant":[42],"hippocampus,":[45],"role":[47],"of":[48,149,193,207],"this":[49],"region":[50],"remains":[53],"relatively":[54],"understudied.":[55],"Here":[56],"we":[57,172],"characterize":[58],"hippocampal":[59,82,133,140],"function":[60],"across":[61],"scales":[62],"mice":[64,159],"carrying":[65],"patient":[67],"orthologous":[68],"Wfs1":[69],"variant":[70],"(p.W542*)":[71],"on":[72],"both":[73],"alleles.":[74],"Male":[75],"mutants":[76],"lose":[77],"excitatory":[78,194],"synapses,":[79],"dissociated":[81],"neurons":[83],"are":[84],"markedly":[85],"hyperexcitable":[86],"yet":[87],"generate":[88],"no":[89,95,123,132],"increase":[90],"network":[92,98,186],"bursting":[93],"shift":[96],"criticality,":[99],"indicating":[100],"that":[101,174],"their":[102],"additional":[103],"firing":[104],"not":[106,182],"recruited":[107],"into":[108,184],"coordinated":[109],"population":[110],"activity.":[111,169],"This":[112,196],"finding":[113],"translates":[114],"vivo":[116],"wherein":[117],"intracranial":[118],"electroencephalogram":[119],"(EEG)":[120],"recordings":[121],"show":[122,160],"epileptiform":[124],"activity":[125],"or":[126],"seizures":[127],"either":[129],"sex,":[130],"with":[131],"astrogliosis,":[134],"alongside":[135],"male-specific":[137],"reduction":[138],"low-gamma":[141],"power.":[142],"Additionally,":[143],"EEG":[144],"demonstrates":[145],"sex-specific":[147],"disruption":[148],"sleep,":[150],"which":[151],"parallels":[152],"sex-divergent":[154],"synaptic":[155],"phenotype.":[156],"Mutant":[157],"male":[158],"impaired":[161],"hippocampus-dependent":[162],"fear":[163],"memory":[164],"reduced":[166],"voluntary":[167],"motor":[168],"Taken":[170],"together,":[171],"propose":[173],"cellular":[175],"hyperexcitability":[176],"hippocampus":[180],"does":[181],"translate":[183],"increased":[185],"bursting,":[187],"potentially":[188],"due":[189],"to":[190],"disproportionate":[191],"loss":[192],"synapses.":[195],"dissociation":[197],"may":[198],"provide":[199],"functional":[201],"substrate":[202],"for":[203],"features":[206],"WS.":[208]},"counts_by_year":[],"updated_date":"2026-11-09T13:22:03.289127","created_date":"2026-10-03T00:00:00"}
