{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,2,21]],"date-time":"2026-02-21T14:34:21Z","timestamp":1771684461552,"version":"3.50.1"},"reference-count":40,"publisher":"Georg Thieme Verlag KG","issue":"09","funder":[{"name":"Japanese Ministry of Health, Labor, and Welfare","award":["21FC1008"],"award-info":[{"award-number":["21FC1008"]}]},{"DOI":"10.13039\/100009619","name":"Japan Agency for Medical Research and Development","doi-asserted-by":"crossref","award":["JP16ek0109043"],"award-info":[{"award-number":["JP16ek0109043"]}],"id":[{"id":"10.13039\/100009619","id-type":"DOI","asserted-by":"crossref"}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Thromb Haemost"],"published-print":{"date-parts":[[2023,9]]},"abstract":"<jats:p>\n            Background\u2003Coagulation factor XIII (FXIII) is a proenzyme of plasma transglutaminase. It comprises two catalytic A subunits (FXIII-A) and two carrier B subunits (FXIII-B). We previously reported that alloantibodies against FXIII-B could promote FXIII clearance in a patient with congenital FXIII-B deficiency who had received infusions of plasma-derived human FXIII (A2B2 heterotetramer).<\/jats:p><jats:p>\n            Objectives\u2003We aimed to investigate whether anti-FXIII-B antibodies affect the catalytic function of FXIII.<\/jats:p><jats:p>\n            Methods\u2003FXIII activation and fibrin crosslinking were examined in the presence of patient plasma, isolated patient IgG, or rat anti-FXIII-B monoclonal antibodies.<\/jats:p><jats:p>\n            Results\u2003Alloantibody levels were increased by repeated infusions of plasma-derived A2B2 heterotetramer, which enhanced binding to the functionally important FXIII-B sushi domains. The patient plasma strongly inhibited cleavage of the FXIII-A activation peptide, amine incorporation, and fibrin crosslinking in normal plasma. Furthermore, anti-FXIII-B alloantibodies blocked the formation of the complex of FXIII-B with FXIII-A, and fibrinogen. Rat monoclonal antibodies against the 10th sushi domain of FXIII-B inhibited the incorporation of FXIII-B to fibrin, FXIII activation (i.e., cleavage of FXIII-A activation peptide), and ultimately fibrin crosslinking in normal plasma, independent of their effect on heterotetramer assembly with FXIII-A. Alloantibody binding to the A2B2 heterotetramer blocked the access of thrombin to the FXIII-A cleavage site, as indicated by the reaction of the alloantibodies to the A2B2 heterotetramer and FXIII-B, but not to FXIII-A.<\/jats:p><jats:p>\n            Conclusion\u2003Anti-FXIII-B antibodies binding to the A2B2 heterotetramer and FXIII-B inhibited FXIII activation and its crosslinking function despite being directed against its noncatalytic subunit (FXIII-B).<\/jats:p>","DOI":"10.1055\/a-2057-8710","type":"journal-article","created":{"date-parts":[[2023,3,19]],"date-time":"2023-03-19T23:30:25Z","timestamp":1679268625000},"page":"841-854","source":"Crossref","is-referenced-by-count":7,"title":["Antibodies against Noncatalytic B Subunit of Factor XIII Inhibit Activation of Factor XIII and Fibrin Crosslinking"],"prefix":"10.1055","volume":"123","author":[{"given":"Masayoshi","family":"Souri","sequence":"additional","affiliation":[{"name":"Department of Molecular Patho-Biochemistry and Patho-Biology, Yamagata University School of Medicine, Yamagata, Japan"},{"name":"The Japanese Collaborative Research Group (JCRG) on Autoimmune Acquired Coagulation Factor Deficiencies supported by the Japanese Ministry of Health, Labor and Welfare (MHLW), Yamagata, Japan"},{"name":"Department of Public Health and Hygiene, Yamagata University Graduate School of Medical Science, Yonezawa, Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Chikako","family":"Yokoyama","sequence":"additional","affiliation":[{"name":"The Japanese Collaborative Research Group (JCRG) on Autoimmune Acquired Coagulation Factor Deficiencies supported by the Japanese Ministry of Health, Labor and Welfare (MHLW), Yamagata, Japan"},{"name":"Department of Biochemical Engineering, Graduate School of Science and Engineering, Yamagata University, Yamagata, Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Tsukasa","family":"Osaki","sequence":"additional","affiliation":[{"name":"Department of Molecular Patho-Biochemistry and Patho-Biology, Yamagata University School of Medicine, Yamagata, Japan"},{"name":"The Japanese Collaborative Research Group (JCRG) on Autoimmune Acquired Coagulation Factor Deficiencies supported by the Japanese Ministry of Health, Labor and Welfare (MHLW), Yamagata, Japan"},{"name":"Department of Public Health and Hygiene, Yamagata University Graduate School of Medical Science, Yonezawa, Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-3807-5628","authenticated-orcid":false,"given":"Akitada","family":"Ichinose","sequence":"additional","affiliation":[{"name":"Department of Molecular Patho-Biochemistry and Patho-Biology, Yamagata University School of Medicine, Yamagata, Japan"},{"name":"The Japanese Collaborative Research Group (JCRG) on Autoimmune Acquired Coagulation Factor Deficiencies supported by the Japanese Ministry of Health, Labor and Welfare (MHLW), Yamagata, Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"194","published-online":{"date-parts":[[2023,4,19]]},"reference":[{"issue":"15","key":"ref1","doi-asserted-by":"crossref","first-page":"7296","DOI":"10.1073\/pnas.91.15.7296","article-title":"Three-dimensional structure of a transglutaminase: human blood coagulation factor XIII","volume":"91","author":"V C Yee","year":"1994","journal-title":"Proc Natl Acad Sci U S A"},{"issue":"33","key":"ref2","doi-asserted-by":"crossref","first-page":"8656","DOI":"10.1021\/bi8006143","article-title":"Sushi domains in the B subunit of factor XIII responsible for oligomer assembly","volume":"47","author":"M Souri","year":"2008","journal-title":"Biochemistry"},{"issue":"23","key":"ref3","doi-asserted-by":"crossref","first-page":"13411","DOI":"10.1016\/S0021-9258(18)77358-4","article-title":"Structure 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Lorand","year":"1999","journal-title":"Blood"},{"issue":"03","key":"ref38","doi-asserted-by":"crossref","first-page":"723","DOI":"10.1182\/blood-2008-09-179333","article-title":"Severe bleeding complications caused by an autoantibody against the B subunit of plasma factor XIII: a novel form of acquired factor XIII deficiency","volume":"113","author":"E Ajzner","year":"2009","journal-title":"Blood"},{"issue":"02","key":"ref39","doi-asserted-by":"crossref","first-page":"255","DOI":"10.1016\/j.jtha.2022.11.019","article-title":"Cloning of human anti-factor XIII monoclonal antibody dissects mechanisms of polyclonal antibodies in a single patient","volume":"21","author":"M Souri","year":"2023","journal-title":"J Thromb Haemost"},{"issue":"05","key":"ref40","doi-asserted-by":"crossref","first-page":"737","DOI":"10.1111\/jth.14412","article-title":"Factor XIII topology: organization of B subunits and changes with activation studied with single-molecule atomic force microscopy","volume":"17","author":"A D Protopopova","year":"2019","journal-title":"J Thromb Haemost"}],"container-title":["Thrombosis and Haemostasis"],"original-title":[],"language":"en","link":[{"URL":"http:\/\/www.thieme-connect.de\/products\/ejournals\/pdf\/10.1055\/a-2057-8710.pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,8,28]],"date-time":"2023-08-28T22:32:59Z","timestamp":1693261979000},"score":1,"resource":{"primary":{"URL":"http:\/\/www.thieme-connect.de\/DOI\/DOI?10.1055\/a-2057-8710"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2023,3,19]]},"references-count":40,"journal-issue":{"issue":"09","published-online":{"date-parts":[[2023,8,28]]},"published-print":{"date-parts":[[2023,9]]}},"URL":"https:\/\/doi.org\/10.1055\/a-2057-8710","archive":["Portico","CLOCKSS"],"relation":{},"ISSN":["0340-6245","2567-689X"],"issn-type":[{"value":"0340-6245","type":"print"},{"value":"2567-689X","type":"electronic"}],"subject":[],"published-other":{"date-parts":[[2023,3,19]]},"published":{"date-parts":[[2023,3,19]]}}}