{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,6]],"date-time":"2026-10-06T08:48:06Z","timestamp":1791276486497,"version":"4.1.0"},"reference-count":40,"publisher":"Oxford University Press (OUP)","issue":"1","license":[{"start":{"date-parts":[[2021,12,20]],"date-time":"2021-12-20T00:00:00Z","timestamp":1639958400000},"content-version":"vor","delay-in-days":2846,"URL":"https:\/\/creativecommons.org\/licenses\/by\/3.0\/"}],"funder":[{"DOI":"10.13039\/501100001700","name":"Ministry of Education, Culture, Sports, Science and Technology","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100001700","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100001691","name":"Japan Society for the Promotion of Science","doi-asserted-by":"publisher","award":["00285024"],"award-info":[{"award-number":["00285024"]}],"id":[{"id":"10.13039\/501100001691","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2014,3,6]]},"abstract":"<jats:title>Summary<\/jats:title><jats:p>Uncontrolled activation of the complement alternative pathway is associated with complement-mediated renal disease. Factor B and factor D are essential components of this pathway, while factor H (FH) is its major regulator. In complete FH deficiency, uncontrolled C3 activation through the alternative pathway results in plasma C3 depletion and complement-mediated renal disease. These are dependent on factor B. Mannan-binding lectin-associated serine proteases 1 and 3 (MASP-1, MASP-3) have been shown recently to contribute to alternative pathway activation by cleaving pro-factor D to its active form, factor D. We studied the contribution of MASP-1 and MASP-3 to uncontrolled alternative pathway activation in experimental complete FH deficiency. Co-deficiency of FH and MASP-1\/MASP-3 did not ameliorate either the plasma C3 activation or glomerular C3 accumulation in FH-deficient mice. Our data indicate that MASP-1 and MASP-3 are not essential for alternative pathway activation in complete FH deficiency.<\/jats:p>","DOI":"10.1111\/cei.12244","type":"journal-article","created":{"date-parts":[[2013,11,27]],"date-time":"2013-11-27T04:23:36Z","timestamp":1385526216000},"page":"84-92","source":"Crossref","is-referenced-by-count":32,"title":["C3 dysregulation due to factor H deficiency is mannan-binding lectin-associated serine proteases (MASP)-1 and MASP-3 independent<i>in vivo<\/i>"],"prefix":"10.1093","volume":"176","author":[{"given":"M M","family":"Ruseva","sequence":"first","affiliation":[{"name":"Centre for Complement & Inflammation Research, Imperial College London, London, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"M","family":"Takahashi","sequence":"additional","affiliation":[{"name":"Department of Immunology, Fukushima Medical University School of Medicine, Fukushima, Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"T","family":"Fujita","sequence":"additional","affiliation":[{"name":"Department of Immunology, Fukushima Medical University School of Medicine, Fukushima, Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"M C","family":"Pickering","sequence":"additional","affiliation":[{"name":"Centre for Complement & Inflammation Research, Imperial College London, London, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"286","published-online":{"date-parts":[[2014,3,6]]},"reference":[{"key":"2021122010020563900_cei12244-bib-0001","doi-asserted-by":"crossref","first-page":"333","DOI":"10.1016\/j.sbi.2012.04.001","article-title":"The modular serine proteases of the complement cascade","volume":"22","author":"Forneris","year":"2012","journal-title":"Curr Opin Struct 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