{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,22]],"date-time":"2026-09-22T11:22:44Z","timestamp":1790076164459,"version":"4.0.1"},"reference-count":35,"publisher":"Oxford University Press (OUP)","issue":"8","license":[{"start":{"date-parts":[[2015,10,1]],"date-time":"2015-10-01T00:00:00Z","timestamp":1443657600000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/academic.oup.com\/pages\/standard-publication-reuse-rights"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2015,10,15]]},"abstract":"<jats:title>Abstract<\/jats:title>\n               <jats:p>C1 inhibitor (C1-INH) is known to form complexes with the lectin complement pathway serine proteases MASP-1 and MASP-2. Deficiency of C1-INH is associated with hereditary angioedema (HAE), an autosomal inherited disease characterized by swelling attacks caused by elevated levels of bradykinin. MASP-1 was shown to cleave high m.w. kininogen into bradykinin; therefore, we hypothesized that MASP-1 levels and the quantity of MASP-1\/C1-INH complexes might be associated with different paraclinical and clinical outcomes of HAE. We measured MASP-1 serum concentrations and endogenous MASP-1\/C1-INH complex levels in 128 HAE patients and 100 controls. Relatively high levels of pre-existing MASP-1\/C1-INH complexes were observed in normal serum, and we found that both the serum levels of MASP-1 and the complex formation between MASP-1 and C1-INH were significantly reduced in HAE patients compared with matched controls (p &amp;lt; 0.0001). The level of MASP-1 and MASP-1\/C1-INH complexes in HE patients correlated with the level of C1-INH (p = 0.0009 and p = 0.0047, respectively), the level of C4 (p = 0.0084 and p &amp;lt; 0.0001, respectively), and the number of attacks in the year of blood sampling (p = 0.0075 and p = 0.0058, respectively). In conclusion, we show that MASP-1\/C1-INH complexes circulate in normal human blood. The levels of MASP-1 and MASP-1\/C1-INH complexes are reduced in HAE patients compared with controls. Both MASP-1 and MASP-1\/C1-INH complexes are related to the degree of complement C4 consumption, as well as the severity of disease. These results suggest that MASP-1 may exert a previously unrecognized role in the pathophysiology of HAE.<\/jats:p>","DOI":"10.4049\/jimmunol.1402838","type":"journal-article","created":{"date-parts":[[2015,9,15]],"date-time":"2015-09-15T01:40:59Z","timestamp":1442281259000},"page":"3596-3604","source":"Crossref","is-referenced-by-count":43,"title":["The Levels of the Lectin Pathway Serine Protease MASP-1 and Its Complex Formation with C1 Inhibitor Are Linked to the Severity of Hereditary Angioedema"],"prefix":"10.1093","volume":"195","author":[{"given":"Cecilie Bo","family":"Hansen","sequence":"first","affiliation":[{"name":"Laboratory of Molecular Medicine, Department of Clinical Immunology, Faculty of Health and Medical Sciences, University Hospital of Copenhagen , 2200 Copenhagen ,","place":["Denmark"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Dorottya","family":"Csuka","sequence":"additional","affiliation":[{"name":"3rd Department of Internal Medicine, Semmelweis University , Budapest 1085 ,","place":["Hungary"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Lea","family":"Munthe-Fog","sequence":"additional","affiliation":[{"name":"Laboratory of Molecular Medicine, Department of Clinical Immunology, Faculty of Health and Medical Sciences, University Hospital of Copenhagen , 2200 Copenhagen ,","place":["Denmark"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Lilian","family":"Varga","sequence":"additional","affiliation":[{"name":"3rd Department of Internal Medicine, Semmelweis University , Budapest 1085 ,","place":["Hungary"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Henriette","family":"Farkas","sequence":"additional","affiliation":[{"name":"3rd Department of Internal Medicine, Semmelweis University , Budapest 1085 ,","place":["Hungary"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Karin M\u00f8ller","family":"Hansen","sequence":"additional","affiliation":[{"name":"Laboratory of Molecular Medicine, Department of Clinical Immunology, Faculty of Health and Medical Sciences, University Hospital of Copenhagen , 2200 Copenhagen ,","place":["Denmark"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Claus","family":"Koch","sequence":"additional","affiliation":[{"name":"Department of Cancer and Inflammation, University of Southern Denmark , 5000 Odense ,","place":["Denmark"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Karsten","family":"Skj\u00f8dt","sequence":"additional","affiliation":[{"name":"Department of Cancer and Inflammation, University of Southern Denmark , 5000 Odense ,","place":["Denmark"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Peter","family":"Garred","sequence":"additional","affiliation":[{"name":"Laboratory of Molecular Medicine, Department of Clinical Immunology, Faculty of Health and Medical Sciences, University Hospital of Copenhagen , 2200 Copenhagen ,","place":["Denmark"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mikkel-Ole","family":"Skjoedt","sequence":"additional","affiliation":[{"name":"Laboratory of Molecular Medicine, Department of Clinical Immunology, Faculty of Health and Medical Sciences, University Hospital of Copenhagen , 2200 Copenhagen ,","place":["Denmark"]}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"286","published-online":{"date-parts":[[2015,10,15]]},"reference":[{"key":"2025030704183688400_r1","doi-asserted-by":"crossref","first-page":"1058","DOI":"10.1056\/NEJM200104053441406","article-title":"Complement. 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