{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,5,29]],"date-time":"2026-05-29T20:15:44Z","timestamp":1780085744891,"version":"3.54.0"},"reference-count":28,"publisher":"Wiley","issue":"2","license":[{"start":{"date-parts":[[2020,1,20]],"date-time":"2020-01-20T00:00:00Z","timestamp":1579478400000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc-nd\/4.0\/"}],"funder":[{"name":"EU Joint Programme - Neurodegenerative Disease Research (JPND) network"},{"name":"PreFrontAls","award":["01ED1512"],"award-info":[{"award-number":["01ED1512"]}]},{"name":"the foundation of the state of Baden-W\u00fcrttemberg","award":["D.3830"],"award-info":[{"award-number":["D.3830"]}]},{"name":"the Thierry Latran foundation"},{"name":"German Federal Ministry of Education and Research","award":["FTLDc 01GI1007A"],"award-info":[{"award-number":["FTLDc 01GI1007A"]}]},{"name":"BIU","award":["D.5009"],"award-info":[{"award-number":["D.5009"]}]},{"name":"Italian Ministry of Health","award":["Ricerca corrente"],"award-info":[{"award-number":["Ricerca corrente"]}]}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Ann Clin Transl Neurol"],"published-print":{"date-parts":[[2020,2]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Objective<\/jats:title><jats:p>SerpinA1 (alpha\u20101 antitrypsin) is an acute inflammatory protein, which seems to play a role in neurodegeneration and neuroinflammation. In Alzheimer\u2019s disease and synucleinopathies, SerpinA1 is overexpressed in the brain and the cerebrospinal fluid (CSF) showing\u00a0abnormal patterns of its charge isoforms. To date, no comprehensive studies explored SerpinA1 CSF isoforms in Creutzfeldt\u2013Jakob disease (CJD) and frontotemporal lobar degeneration (FTLD).<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>Using a capillary isoelectric focusing immunoassay, we analyzed CSF SerpinA1 isoforms in control cases (<jats:italic>n<\/jats:italic>\u00a0=\u00a031) and patients with a definite or probable diagnosis of\u00a0CJD (n=77) or FTLD (<jats:italic>n<\/jats:italic>\u00a0=\u00a030), belonging to several disease subtypes.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>The overall SerpinA1 signal was significantly higher than in controls in CJD subtypes linked to abnormal prion protein (PrP<jats:sup>Sc<\/jats:sup>) type 1, such as sporadic CJD (sCJD) MM(V)1, and in FTLD\u2010TDP. Moreover, CJD linked to PrP<jats:sup>Sc<\/jats:sup> type 1 and FTLD\u2010TAU groups showed a significant relative increase of acidic and basic isoforms in comparison with controls, thereby forming two distinct SerpinA1 isoform profiles.<\/jats:p><\/jats:sec><jats:sec><jats:title>Interpretation<\/jats:title><jats:p>CJD linked to PrP<jats:sup>Sc<\/jats:sup> type 1 and FTLD show a differential upregulation and post\u2010translational modifications of CSF SerpinA1. Further studies are needed to clarify whether these findings may reflect a common, albeit disease\u2010specific, pathogenetic mechanism related to neurodegeneration.<\/jats:p><\/jats:sec>","DOI":"10.1002\/acn3.50980","type":"journal-article","created":{"date-parts":[[2020,1,20]],"date-time":"2020-01-20T09:15:01Z","timestamp":1579511701000},"page":"191-199","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":25,"title":["CSF SerpinA1 in Creutzfeldt\u2013Jakob disease and frontotemporal lobar degeneration"],"prefix":"10.1002","volume":"7","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-0631-8506","authenticated-orcid":false,"given":"Samir","family":"Abu\u2010Rumeileh","sequence":"first","affiliation":[{"name":"Department of Biomedical and NeuroMotor Sciences (DIBINEM) University of Bologna 40139 Bologna 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