{"doi":"10.1073/pnas.2402983121","title":"A sensitive assay for measuring whole-blood responses to type I IFNs","abstract":"Human inborn errors of the type I IFN response pathway and auto-Abs neutralizing IFN-α, -β, and/or -ω can underlie severe viral illnesses. We report a simple assay for the detection of both types of condition. We stimulate whole blood from healthy individuals and patients with either inborn errors of type I IFN immunity or auto-Abs against type I IFNs with glycosylated human IFN-α2, -β, or -ω. As controls, we add a monoclonal antibody (mAb) blocking the type I IFN receptors and stimulate blood with IFN-γ (type II IFN). Of the molecules we test, IP-10 (encoded by the interferon-stimulated gene (ISG) CXCL10 ) is the molecule most strongly induced by type I and type II IFNs in the whole blood of healthy donors in an ELISA-like assay. In patients with inherited IFNAR1, IFNAR2, TYK2, or IRF9 deficiency, IP-10 is induced only by IFN-γ, whereas, in those with auto-Abs neutralizing specific type I IFNs, IP-10 is also induced by the type I IFNs not neutralized by the auto-Abs. The measurement of type I and type II IFN-dependent IP-10 induction therefore constitutes a simple procedure for detecting rare inborn errors of the type I IFN response pathway and more common auto-Abs neutralizing type I IFNs.","journal":"Proceedings of the National Academy of Sciences","year":2024,"id":427082,"datarank":0.44166584687496613,"base_score":2.9444389791664403,"endowment":2.9444389791664403,"self_citation_contribution":0.44166584687496613,"citation_network_contribution":0.0,"self_endowment_contribution":0.44166584687496613,"citer_contribution":0.0,"corpus_percentile":null,"corpus_rank":null,"citation_count":18,"citer_count":0,"citers_with_citation_signal":0,"citers_with_endowment":0,"datacite_reuse_total":0,"is_dataset":false,"is_dataset_confidence":0.9648,"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":1028526,"name":"Corentin Le Floc’h","orcid":"0009-0007-0590-0437","position":1,"is_corresponding":false},{"id":618891,"name":"Tom Le Voyer","orcid":"0000-0002-7253-3135","position":2,"is_corresponding":false},{"id":104153,"name":"Lucy Bizien","orcid":"0000-0001-9163-9122","position":3,"is_corresponding":false},{"id":839708,"name":"Jonathan Bohlen","orcid":"0000-0003-0458-9484","position":4,"is_corresponding":false},{"id":655788,"name":"Fatih Çelmeli","orcid":"0000-0002-2983-5058","position":5,"is_corresponding":false},{"id":730018,"name":"Fahd Al Qureshah","orcid":"0000-0002-5022-9917","position":6,"is_corresponding":false},{"id":104486,"name":"Cécile Masson","orcid":"0000-0001-7870-7821","position":7,"is_corresponding":false},{"id":104146,"name":"Jérémie Rosain","orcid":"0000-0002-2822-161X","position":8,"is_corresponding":false},{"id":104473,"name":"Marwa Chbihi","orcid":"0000-0002-2771-851X","position":9,"is_corresponding":false},{"id":273547,"name":"Romain Lévy","orcid":"0000-0002-6393-0198","position":10,"is_corresponding":false},{"id":104210,"name":"Riccardo Castagnoli","orcid":"0000-0003-0029-9383","position":11,"is_corresponding":false},{"id":618899,"name":"Anya Rothenbühler","orcid":"0000-0001-9246-5160","position":12,"is_corresponding":false},{"id":104229,"name":"Emmanuelle Jouanguy","orcid":"0000-0002-7358-9157","position":13,"is_corresponding":false},{"id":104140,"name":"Qian Zhang","orcid":"0000-0002-9040-3289","position":14,"is_corresponding":false},{"id":57374,"name":"Shen-Ying Zhang","orcid":"0000-0002-9449-3672","position":15,"is_corresponding":false},{"id":104147,"name":"Vivien Béziat","orcid":"0000-0002-4020-824X","position":16,"is_corresponding":false},{"id":241304,"name":"Jacinta Bustamante","orcid":"0000-0002-3439-2482","position":17,"is_corresponding":false},{"id":104226,"name":"Anne Puel","orcid":"0000-0003-2603-0323","position":18,"is_corresponding":false},{"id":104138,"name":"Paul Bastard","orcid":"0000-0002-5926-8437","position":19,"is_corresponding":false},{"id":57373,"name":"Jean-Laurent Casanova","orcid":"0000-0002-7782-4169","position":20,"is_corresponding":false},{"id":615375,"name":"Adrian Gervais","orcid":"0000-0002-1083-5787","position":0,"is_corresponding":true}],"reference_count":105,"raw_metadata":{"citation_network_status":"fetched"},"created_at":"2026-07-19T01:58:47.805440Z","pmid":"39312669","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":[]}