{"doi":"10.1016/j.brainres.2024.149082","title":"Hypersensitivity to type I interferon as a cause of hydrocephalus development","abstract":"• The depletion of IFN receptor diminished hydrocephalus of Usp18 deficient mice. • USP18-depleted ependymal cells exhibit heightened responsiveness to type I IFN stimulation. • The hyperactive type I IFN response observed in USP18-depleted ependymal cells leads to cell death, including pyroptosis. Ubiquitin specific protease 18 (USP18) serves as a potent inhibitor of Type I interferon (IFN) signaling. Previous studies have shown that Usp18 deficient (homozygous Usp18 gene knockout) mice exhibit hydrocephalus; however, the precise molecular mechanism underlying hydrocephalus development remains elusive. In this study, we demonstrate that mice lacking both type I IFN receptor subunit 1 ( Ifnar1 ) and Usp18 ( Ifnar1 / Usp18 double knockout mice) are viable and do not display a hydrocephalus phenotype. Moreover, we observed that suppression of USP18 in ependymal cells treated with IFN significantly increased cell death, including pyroptosis, and decreased proliferation. These findings suggest that heightened sensitivity to type I IFN during brain development contributes to the onset of hydrocephalus. Furthermore, they imply that inhibition of IFN signaling may hold promise as a therapeutic strategy for hydrocephalus.","journal":"Brain Research","year":2024,"id":498334,"datarank":0.0,"base_score":0.0,"endowment":0.0,"self_citation_contribution":0.0,"citation_network_contribution":0.0,"self_endowment_contribution":0.0,"citer_contribution":0.0,"corpus_percentile":null,"corpus_rank":null,"citation_count":0,"citer_count":0,"citers_with_citation_signal":0,"citers_with_endowment":0,"datacite_reuse_total":0,"is_dataset":false,"is_dataset_confidence":0.9545,"is_data_producer":false,"deposit_databanks":null,"is_oa":true,"file_count":0,"downloads":0,"has_version_chain":false,"published_date":"2024-01-01","fair_score":null,"fair_percentile":null,"algorithm_id":"datarank_citation_only_1hop_v6","ranking_scope":"data_only","authors":[{"id":659967,"name":"Yue Zhang","orcid":"0000-0002-2955-7780","position":1,"is_corresponding":false},{"id":354208,"name":"Shinobu Matsuura","orcid":"0000-0002-3914-5376","position":2,"is_corresponding":false},{"id":312929,"name":"Sayuri Miyauchi","orcid":null,"position":3,"is_corresponding":false},{"id":311115,"name":"Dong‐Er Zhang","orcid":"0000-0003-2541-6443","position":4,"is_corresponding":false},{"id":312931,"name":"Kei‐ichiro Arimoto","orcid":null,"position":0,"is_corresponding":true}],"reference_count":22,"raw_metadata":null,"created_at":"2026-07-19T02:09:38.543544Z","pmid":"38866307","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":[]}